today at five, the latest on the severe weather causing disruption across the uk. hundreds of schools have been closed and thousands of households have no power, as snow and ice cover much of the uk. temperatures tonight may plunge to —12. we'll have the latest from different parts of the uk as rush hour traffic develops and public transport is badly affected. the other main stories on bbc news at five... police in manchester launch a murder investigation after three children die in a house fire. the mayor of new york says an explosion in central manhattan was an "attempted terror attack". the suspect was injured and is now in custody. scientists trying to find a treatment for huntington's disease think they may be close to the biggest breakthrough for 50 years. the broadcaster, keith chegwin, one of the familiar faces of children's television in the 1980s, has died at the age of 60 after a long illness. it's five o'clock.

our main story is that snow and freezing temperatures are causing widespread disruption in many parts of the uk. heathrow airport is trying to clear a backlog of hundreds of flights, the port of calais is closed, while roads and rail have been badly affected. it's all making travel difficult and even hazardous. more than a thousand schools failed to open this morning because of the snow. hundreds of homes, mainly in wales, and a belt across the centre of england, are without power. the forecasters say temperatures could fall in places tonight to as low as —12 celsius. our correspondent phil mackie reports. a large swathe of central england and most of wales woke up

to a winter wonderland, but the morning commute was tricky, many areas saw their heaviest snowfall for seven years. some areas saw 20 centimetres, which froze when temperatures plummeted. driving conditions were extremely dangerous, and the breakdown services have been working around the clock. on an average sunday, we do 10,000 breakdowns, but yesterday we did 1a,000 — 600 of those were vehicles stuck in snow. the main advice would be to take it very steady, pack a few extras in the car, like a blanket. if you have a torch, a mobile phone, make sure they are charged. make sure you know how to use everything safely. make sure you have your main contacts saved in your phone should you need them desperately. there's been criticism of local authorities after hundreds of schools were shut, meaning an enforced day off for parents who had to look after children. as you can see, for a lot of people, it is a snow day, a lot of fun, but it's been a real problem for businesses especially because people cannot get to work,

in some places they have had to stay at home to look after children. for others, simply because the high street should be doing quite well in the run—up to christmas but this is keeping people away. a lot of snow and ice around, it will stay like this for at least another day. the snowploughs and gritters have been working at full pelt throughout the weekend. for many areas, this is day four of disruption. the worst conditions are over the higher ground in england and wales, where they are more used to it and quite phlegmatic. happens every four or five years, we geta big dump, and obviously being higher up, we tend to get more than most, but it is very pretty. everyone is being asked to help out, including organisations with four—wheel drive vehicles. we offered up a number of them to our health colleagues, because there is a lot of pressure on them at the moment. getting people to patients, patients to hospitals. we put them at the disposal of our health colleagues. we have well—trained drivers.

we have good vehicles we are happy to lend. the cold snap is not yet over, that means there is no prospect of an early thaw, so the big question is, what happens next? across the country, we have had a lot of snow and also rain and sleet over the last 2a to 48 hours. tonight, temperatures will plunge, in some spots, —10, —11, —12 degrees. with all those wet and snowy surfaces, there could be some severe ice problems to take us into tomorrow morning. whether you love it or hate it, it is undeniably pretty, and social media is filled with pictures like this. so, this year, many people are enjoying some stunning scenery, and christmas come early. phil mackie, bbc news, worcestershire. let's get an update. we will be talking to simon jones let's get an update. we will be talking to simonjones in dover in a moment about all the transport affected today. first, we are

speaking to a correspondent at a gritting depot. the guys here have been flat out for days when temperatures for strop. tonight could be the busiest night so tonight could be the busiest night so far. temperatures in gloucestershire to get down to —12 celsius. that is the air temperature. the low temperature could be minus nine celsius. they are working out when to send out the gritters. 400 tonnes of grit and salt will be going onto the road tonight. they have to make sure the ground is wet enough to soak up the salt and keep doing a good job. they have to time to avoid the worst of the traffic congestion so as not to cause more delays. it is important before it gets even colder because it might not be effective. they are worrying about the timing, trying to

get it right. 450 tonnes to go out just in this county. that will be the equivalent... the amount of salt we put on 300 million fish and chip portions. if anyone is having fish and chips 40, a margin of 300 million identical portions. that much salt. that is just in gloucestershire. madge and how much salt around the country to make. gloucestershire. madge and how much salt around the country to makem is quite an image for us to conjure up is quite an image for us to conjure up will stop i accept that. i wonder, resource wise... lots of local authorities are feeling the pinch and they are certainly kidding pennies in terms of budgets. what does this kind of operation been in terms of the resorts pressure? —— the resource. there are strict laws about how much time drivers can spend out there. they have to do a certain shift and then have a break. i spoke to one gentleman who work here who said he had only been home for a matter of three or four hours since the beginning of the "to catch up since the beginning of the "to catch up on sleep on the car and that kind of thing just to make sure that they

are onjt of thing just to make sure that they are oan and they are aware that they can get out and do the job. in gloucestershire, this is on the fringes of the worst affected area of the whole uk. it is notjust this cou nty of the whole uk. it is notjust this county dealing with it, it is many other counties and local authorities across the country. the guys here in this council facility are not looking after the m5 motorway. that isa looking after the m5 motorway. that is a different network and different area of responsibility. it is a big, coordinated assembly of organisation. it takes a lot of people power and hours. they hope tomorrow it might warm up a bit salt will not require the intensity of effort that it has in the last few days. but the last four days have put them to the stretch and they need respite quick. thank you for the update with all the work going on there. simon, in dover, what is going on there? we havejust

on there. simon, in dover, what is going on there? we have just heard that in the last few minutes, the port of calais has now reopened, having been shut for around three hours this afternoon. yet in dover, we have had snow during the course of the afternoon. now it has turned to the freezing rain that really soaks into you. 0ver to the freezing rain that really soaks into you. over in france, it has been worse, which is why they decided they could not allow any ferry movements during the course of the afternoon. it is pretty rare for them to decide to shut the board. having said that, it was also shut in calais yesterday. that was largely because of an accident involving a ferry with 300 people on board. the pride of kent. it ran aground in stormy conditions. there is now one investigation under way involving french and british authorities to ascertain what caused that, whether it was solely whether oi’ that, whether it was solely whether or whether it was to do with error by the captain. that will be looked into. there will be big issues with eurotunnel. 0vernight, passengers

had to wait for seven hours in the freezing cold waiting to get on trains. they were told the train was coming and were given time. then it was another couple of hours and another couple of hours. some people we re another couple of hours. some people were in their carfor another couple of hours. some people were in their car for seven another couple of hours. some people were in their carfor seven hours waiting to cross the channel. it has not been an easy day if you want to travel to france are back. particularly overnight, difficult conditions. inland, problems on the roads. we have our trees down and schools shut. we often say this in situations like this, particularly standing out in the cold and wet, but before you decide to head out, check before you travel, check with your ferry operator, the airports, get that advice before heading out into the freezing conditions. indeed, simon. thank you for that. thank you for the update. travelling from parts of western europe, over to britain, travelling from parts of western europe, overto britain, has travelling from parts of western europe, over to britain, has not been easy. joining me now is james brown.

he has been stranded in luxembourg for over 24 hours after his flight was cancelled. hejoins us now. it is good of he joins us now. it is good of you to talk to us. what is your position now? we got to the airport super early. to make sure we find out if anything else will happen to flight at nine o'clock. when we meant to be flying? nine o'clock last night. what is the information be like for you? awful. i have spoken to fly provider, ryanair. they would not a nswer provider, ryanair. they would not answer phones or web chat last night. we'rejust answer phones or web chat last night. we're just hoping answer phones or web chat last night. we'rejust hoping ourflight goes ahead tonight. how did you get into a position where you could actually get onto a flight this evening? it was london stansted airport. they tweeted that the flight had airport. they tweeted that the flight had been cancelled. we saw there. we managed to get through to a lady from ryanair after about 20 calls last night. she rebooked us

onto this flight. it is a bit convoluted. what other conditions like at the airport? it is ok. it was a bit of a trek today. it has been cold and raining all day. there's not much to do when it is like that. it is a bit of a buzz kill from the whole trip. but we are at the airport super early just hoping we can get a flight home.“ there a chance that you would get on your flight tonight? —— will not get. we will not find out who we get to the gate. they have been overbooked but we are hopeful we should be able to board.|j overbooked but we are hopeful we should be able to board. i do not wa nt to should be able to board. i do not want to be pessimistic, but do you have any plans in case it doesn't work out? exactly the same as today, get a hotel across the road, wake up, waitfor get a hotel across the road, wake up, wait for another flight. hope we get on that flight. you seem

remarkably sanguine, i have to say! we have seen the sites, we have an extra day out of it. hopefully ryanair will reimburse us for our hotel. we shall see! it is good to talk to you. i hope you get away tonight. thank you. james brynin, waiting for that flight in luxembourg to come back to uk. years been waiting 24 hours already. —— he has been. we will keep our fingers crossed for him for that flight this evening. it is 12 minutes past five. and you can keep up to date with the latest disruption on our website at bbc.co.uk/news. if you go there, obviously you will see the range of today's news stories. there is a big section on the weather and that will take you to the bbc weather section, to transport and links about stories

around the uk. have a look at that. the latest news is there for you. let's move onto some of the day ‘s other news. police in greater manchester are treating as murder the deaths of three children in a fire at a house in the worsley area of salford. a 3 year—old girl is critically ill, and two boys of 16 were also wounded. 0ur correspondent alice hutton reports. the fire began shortly before 5am this morning on this terrace near sa lfo rd. this morning on this terrace near salford. a 14—year—old girl was declared dead at the scene. while an eight—year—old boy and seven—year—old girl died later in hospital. italy —year—old girl in critical condition. their mother is see this legal in hospital and unaware that her children have died. —— a three—year—old girl. greater manchester police say that this is being treated as a murder enquiry. the deaths of three children is heartbreaking. 0ur the deaths of three children is heartbreaking. our thoughts are with the family. the little girl and her mum who were fighting for their lives. we have launched a murder

investigation and we have a suspect who we are urgently seeking english into this attack. —— in relation to. he knows who he is and he should give himself up immediately. police are now working with specialist fire and investigators to establish the cause of the blaze. it is a joint agency investigation. we want to identify exactly what was behind this fire, help to assist with any prosecutions following out of it, and they will sift through the debris of the fire. fire investigators are specially trained with the scenes of crime team from manchester police. as the manhunt gets under way, police are appealing for witnesses to come forward. let's get the latest from the scene. danny savage is at there. the latest from police is that they

are being very forthright in state m e nts are being very forthright in statements at the moment. they say that this was murdered by someone starting a fire just before five o'clock this morning. talking to neighbours, they speak very vividly of seeing an orange fireball at the time and then seeing the children being rested and treated by paramedics in the street, which is now all cold and off. there is basically two sides to this investigation. the investigation of the fire, which the fire service are looking at. and some police officers as well. and a hunt for the prime suspect. police saying that they believe they know who was responsible for this. he knows who yea rs responsible for this. he knows who years and they know that they are after him. they appeal for years and they know that they are after him. they appealfor him to give himself up, basically. the sort of ta ke give himself up, basically. the sort of take this on. what they are doing is... there is a lot of work behind the scenes to trace this individual who police believe started this fire. the family and neighbours meanwhile are having to deal with

the horror of it. three children killed in this incident. a fourth child, aged three, critically ill in hospital. the children's mother, aged 35, also seriously ill in hospital. this is something that has wiped out the whole family and caused great concern and belief to everybody here. it is an ongoing investigation. police trying to trace the person they believe is responsible. danny, many thanks for the latest from the scene. this is bbc news at five — the headlines. more than a thousand schools are closed, and hundreds of homes are without power, as parts of the uk continue to suffer from the effects of heavy snow. a murder investigation is launched after three children die in a house fire in manchester — a fourth is critically ill in hospital. a man is being held after an attempted terror attack at new york city's main bus terminal left four injured including the bomber. and in sport, it is a big ask for

antonio conte's chelsea. they will face five—time when as barcelona in the last 16 of the champions league, welcome on face a tricky tie against last season's runners—up, juventus. there would always be drama at old trafford yesterday, but for all the wrong reasons. afterjose mourinho had water and milk run at him, while city's coach suffered a head injury after as row. absurd and amateurism. this is how events were described by claremont, ahead of the rescheduled match with saracens. i will be back with more later. there's been an attempted terror attack on the main bus station in new york, the port authority terminal in central manhattan, during the morning rush hour. the bomber and at least three bystanders were injured in the blast, which the authorities said had been caused by a device based on a pipe bomb.

there was a stampede as counter—terror police and firefighters led hundreds from the terminal, and from subway lines that run under the building. a man's been arrested. this report from our correspondentjon donnison. america's busiest bus station at morning rush—hour. this is cctv that is believed to show the moment the bomb went off. through the smoke, the attacker, who is thought to have been carrying a pipe bomb, can be seen lying injured on the ground. more than 200,000 people use this terminal every day. suddenly, i see a group of people, like 60 people, running like mad. women fell and nobody even stopped to help because the panic was saul scary. —— so

scary. emergency services descended on midtown manhattan, responding to what the authorities now believe was a failed suicide attack that could have been much worse. this was an attempted terrorist attack. thank god the perpetrator did not achieve his ultimate goals. the bomber, who survived but is badly injured, has been identified and is in custody. responding units, and injured 27—year—old male. we have identified him... he had burns and wins to his body. the investigation at the scene indicates this meal was wearing an improvised, low—tech explosive device attached to his body. police will now try to establish whether the bomber was part of any network and will be eager to question him on his motives. for the latest from the scene, we

are joined for the latest from the scene, we arejoined by our for the latest from the scene, we are joined by our correspondent in new york. what more alike can be shared on this incident that happened earlier today? authorities are in the midst of this investigation now. what we do know is that the man is originally believed to be from bangladesh. he came to the united states in 2011 on an immigrant visa with his family and three or four siblings. an immigrant visa with his family and three orfour siblings. he is believed to be a permanent resident that had no criminal than bangladesh. you can see behind me that while this investigation is ongoing, new yorkers already getting at and running again. eighth ave, shut immediately as emergency responders rushed to the scene, has reopened. the port authority behind me and the subways below, where the device actually exploded in a passageway, authorities say that by

evening rush hour, they hope to have it all back up and running. suddenly, there has been a big sigh of relief in new york. —— certainly. new york's governor said it, that an attack on new york's subway is their greatest fear. there is a collective sigh of relief that this was not much worse than it was. again, the perpetrator now in hospital speaking to authorities. new york authorities said he made a statement but did not go into greater detail about what he said. and whether they do believe he is connected to the group that calls itself the islamic state. thank you for the update from new york. 21 minutes past five. survivors and bereaved families have called on the public inquiry into the grenfell tower disaster, in which 71 people died, to be headed by a panel of experts rather than a single retired judge. the current chairman, sir martin moore—bick,

is holding two days of public hearings to set out the future of the inquiry‘s work. victims of the fire say they are already becoming pushed to the sidelines. our home affairs correspondent tom symonds reports from the hearing in central london. the bereaved, the survivors, the people who lived in the shadow of the tower, their supporters. they are not going anywhere. these two days of hearings will set up the way in which the enquiry does its job, gathers the evidence. at first, much of that will come from the police. but the enquiry heard the voices of those who escaped the fire will be vital. to prepare a statement for the enquiry will, for many of them, be difficult and traumatic. and the process of giving oral evidence to the enquiry, equally, if not more, challenging. but we must also try to move promptly and effectively with the aim of learning lessons from this awful event. in order to save the lives of others and in order to ensure something like this can never happen again.

but the survivors and the families of the bereaved have a string of concerns. they are worried, as so—called core participants, that they are already being pushed to the sidelines. watching as experts and lawyers discuss matters of life and death between themselves. hearings will be conducted exclusively by your counsel and you, rendering the core participants passive attendees. able only to give their own evidence if asked, and to suggest questions, but never to ask them. well, we want to ask questions on behalf of our clients. only the enquiry‘s lawyers will question witnesses. it is led by a singlejudge, sir martin moore—bick. the families want him, joined by others, to make up a panel with people better able to represent the social issues the fire revealed. what we're asking for is a panel of experts that represent the community.

that are diverse, but also have the expertise that is required. their suspicion is not helped by the fact that six months on, many survivors have not been rehoused, with 42 families found homes so far. it has been much more complicated and challenging than we initially thought. but i think by the time... you know, we have bought 300 houses and we are getting people rehoused. that will build the trust. it's action rather than words. that's an ongoing process. inquests have described how the 71 victims died. the enquiry‘s job is to decide why. the police, whether anyone is criminally responsible. difficultjobs in themselves, made far more difficult by the trauma this tragedy has left behind. and tom is here with me now. just to reflect on a few of those points... this sense of how the

victims and residents can have a voice in this enquiry. what is the debate over that? the enquiry is concerned that if you allow people so directly affected by the fire to be on so directly affected by the fire to beona so directly affected by the fire to be on a panel that effectively decides what its outcome is, then that will make it not impartial, which is what it has to be. interestingly, the families are not asking for that. they are not asking to be on the panel but they do believe you can find expats in the kinds of social issues they believe g re nfell tower kinds of social issues they believe grenfell tower has thrown up. put them on the panel and that will give them on the panel and that will give the panel a much wider view of the world it wants to look at. we saw today, to be fair, whilst talking to lawyers. they would not have a great sea how this enquiry progresses. given the complexity and layers and this, and sensitivity, there is a question about how it will take.

residents want answers before too long, but how long will it take? residents want answers before too long, but how long will it take ?m is called to take years. every possibility it will take several yea rs possibility it will take several years to get to the final workings of this enquiry. but sir martin has said he wants to produce an interim report, which he said he wanted to be by easter. we know that that could be autumn next year and there is potential for it to slip further. they will look at how the fire started, what caused it to spread, the response by emergency services. they will not just the response by emergency services. they will notjust be looking at, for example, how quickly the fire spread, but they will be looking at the way the cladding system and panels on the tower during its refurbishment, and it contributed. it isa refurbishment, and it contributed. it is a really big job. —— how much it contributed. theresa may has preached the brexit deal reached on

friday as a good deal. theresa may has updated mps on last week's talks that led to the eu agreeing to move the brexit negotiations onto trade. the prime minister talked about "a new sense of optimism" as she addressed the commons this afternoon. she said the uk would leave the eu and and orderly fashion. let's hear some of the debate now. the progress some of the debate now. the progress so far has required give—and—take from the uk and eu to move forward together and that is what we done. of course, nothing is agreed until everything is agreed. but there is, i believe, new sense of optimism in the talks. and ifully i believe, new sense of optimism in the talks. and i fully hope and expect that we will confirm the arrangements i set out today in the european later this week. 18 months on from the referendum result, the prime minister scraped through phase one of the negotiations. scraped through after 18 months. two months

later than planned, with many of the key aspects of phase one is still not clear. this weekend, cabinet members have managed to contradict each other, indeed some have managed to go even further and contradict themselves. last week, we had the humiliating scene of the prime minister being forced out of the original deal by the dup. rushing back to london, the government had to rewrite the agreement so as to reach the dup's approval. we really have to wonder, who is running the uk? |s have to wonder, who is running the uk? is it have to wonder, who is running the uk? is it arlene foster all the right honourable member for maidenhead? she cannot even get her brexit secretary to agree with her. how on earth did you get a good deal that protects jobs, investment and growth in this country? across these benches, complete unanimity in

congratulating the prime minister on securing this agreement. and if i may say, a pitiful performance from the leader of the opposition. i still don't know what he actually welcomes this agreement because he should do. because it is a major step forward. she might want to suspend tribal politics and invite the leader of the opposition and his front bench colleagues to join the leader of the opposition and his front bench colleagues tojoin her negotiating team, since whatever their tactical differences, they agree with are on the fundamentals of brexit and with drawl from the single market and customs union. disastrous as though that may be. the opposition have demonstrated, not today, but the last weeks, a com plete not today, but the last weeks, a complete inconsistency of every point of detail. it is a national disgrace. can i thank the prime minister for her personal devotion

to work to get the text, as she put it today, strengthened in relation to the constitutional integrity of the whole uk. let us talk to vicky young. what did you make of the exchanges and was there more clarity on what was agreed in terms of the border arrangement? i think what was interesting was that if theresa may is getting praise from anna soubry and sarah broadley, then she must feel she's doing something right and she has said it is good for those who voted remain and those who voted leave but that is partly because people are reading into it what they want, in some areas it is deliberately vague and that unanimity can only last so long because at some point the cabinet and the government will have to decide exactly what kind of relationship we have the european

union, how closely aligned we are. when it came to the money, theresa may confirmed that that would be between 35 and £39 billion. she said is conditional on us getting this trade agreement sometime in the future. that is not what phillip hammond has said in the past. on the issue of the irish border, lots of questions on that, that is the area where people are trying to pin down the government, searching questions to the prime minister, what do you mean about being fully alarmed, how can you do that and then be allowed to divert and her answer is we may wa nt to divert and her answer is we may want the same outcomes as a european partners but we do not necessarily have the same rules. there is a form of agreement for now on the conservative side and we will have to see how long that lasts. thank you for the latest from westminster. coming up in the next first and deputy first ministers the breakthrough which could lead to

some kind of treatment to slow down at least huntingdon ‘s disease. it isa at least huntingdon ‘s disease. it is a huge challenge for its santas andi is a huge challenge for its santas and i will be talking to a leading member of the team who has made this stride forward. now it is time for the weather. the weather is calming down but that is not necessarily good news, because more places will have clear skies overnight, more places will have a frost and more places will have a frost and more places will have a frost and more places will have a severe frost and thatis places will have a severe frost and that is likely to lead to some icy conditions very quickly. there was not as much snow falling today, a mixture of rain sleet and snow. a few showers, the ones in northern ireland and scotland will fade away. showers in the west of wales, wintry ones around the eastern coastal counties of england. for most of us it will be dry, clear, it is already frosty, those are the temperatures

in towns and cities. particularly over the snow fields where we could see headline temperatures of minus 13. it will be cold to start the day and potentially icy but for most of us and potentially icy but for most of us it will be a dry day, we will lose those showers from the east coast and import more cloud, some rain in northern ireland, wind is picking up but ahead of it, although we will see some increasing cloud, most we will see some increasing cloud, m ost pla ces we will see some increasing cloud, most places will be dry, sunny and very cold. this is bbc news — the headlines. heading in to rush hour — parts of the uk continue to suffer from the effects of heavy snow — tonight temperatures may plummet to as low as minus 12. three children have died in a house fire in salford and a fourth is in in a critical condition — police have launched a murder inquiry. an explosion in the heart of new york city is being called an ‘attempted terror attack‘. the suspect was injured and is now in custody. tv presenter keith chegwin —

known for hosting swap shop and cheggers plays pop — has died aged 60 after a long illness... holly is at the bbc sport centre.... we will start with the champions league and chelsea will have to find a way past five—time champions barcelona for the sixth time in the knockout stages and it will not be easy for tottenham, they will have to deal with juventus. easy for tottenham, they will have to deal withjuventus. let's look at the drop. manchester city were handed potentially the most straightforward task, paired with basil and manchester united will fa ncy basil and manchester united will fancy their chances against sevilla with the second leg due to take place at old trafford and liverpool are up against porto. they will be reasonably happy with the drawers but chelsea looked to have the

toughest task stop every game against chelsea and barcelona has been a great game. we have had recent semi—finals against each other, some have been controversial and others not and our last time in the new camp was the semifinal, the year that we went on to munich and in the previous semifinal, any yesterday scored a late goal to clinch it for barcelona. every game that we play against them, it is exciting. we were expecting drama at old trafford yesterday but it was the actions of players and staff after city's win that caused a stir. the football association has asked both clubs for more information after a fracas broke out outside the changing rooms. it is believed jose mourinho was unhappy with that supposedly excessive celebrations by manchester city. one of the city coaches got a cut on his face. jose mourinho sought the manchester city

dressing room was open and basically made his feelings known, there was a reaction, there was a lot of jostling, mikael arteta, and manchester city coach got a cut above his eye, there were some milk throne, it hit a member of the manchester united star. it was all a bit unseemly, the fa have now decided to step in and asked both clu bs decided to step in and asked both clubs what happened, it is outside the jurisdiction clubs what happened, it is outside thejurisdiction of clubs what happened, it is outside the jurisdiction of the referees of the jurisdiction of the referees of the clubs have until the 13th of december to let the fa know what went on. cricket and jonny bairstow says that the england players have listened to the warning of trevor bayliss that players will pay with their places in the discipline of their places in the discipline of the field does not improve. they trail in the ashes and it has been dogged by several of the field incidents. people have perceptions and will read things. incidents. people have perceptions and will read thingslj incidents. people have perceptions and will read things. i firmly

believe that there has been a lot of hype and it has been blown up and it has escalated a heck of a lark. we are ina has escalated a heck of a lark. we are in a position hopefully with the results we have got, we can do the talking that has been escalated off the field. we have an opportunity to do that on the field. we need to get ourselves out of it and we need to win three test matches, that is the bottom line, there is no hiding from that. that is the challenge that we have now got in front of us. finally, the rearranged champions cup match between saracens and clermont auvergne has got under way, that match had been postponed due to the snow and there is live commentary on five live if you want to keep up with bad and you can find out more on that and all the stories today on our website. we will have more fuel in sports day. we will see you later. more than 8,000 people in the uk have

huntington's disease a devastating condition which permanently affects their ability to move and think, as well as how they behave. it's a neuro—degenerative disease which often starts to affect people in their 30s and 40s. and now scientists think they may have made a big breakthrough, which could ultimately stop the deadly disease. we're not quite at that stage. well professor sarah tabrizi led the trial. she is director of university college london's huntington's disease centre. fantastic to have you with us. many congratulations on what people recognise is a significant step forward , recognise is a significant step forward, it is a great day for you and yourchain. forward, it is a great day for you and your chain. how would you describe to viewers what the significance of this step is? huntingdon's disease is caused by a defective gene that causes the build—up ofa defective gene that causes the build—up of a toxic protein in the brains of patients that kills their

nerve cells. the significance of this finding is that a drug has been shown to lower the levels of the mutant proteins in the nervous system of patients affected by huntingdon's disease. this drug in the trial was infused into the spinal fluid the trial was infused into the spinalfluid where the the trial was infused into the spinal fluid where the drug reached the brain and lowered the levels of the brain and lowered the levels of the toxic protein and so what was important about the study, in a small number of people, we were able to show that the drug, the aso drug which was developed, and exciting therapy, we found it was safe, it was well tolerated in its first in man study and we could target the protein that causes the disease and lowered in the nervous system of patience. so, that is the step, i am wondering, where do you go next? is it to do with a much bigger trial or what is it? very important question.

this is the beginning, we know we can target the cause and the next step is to do a much larger trial in hundreds of patients who have symptoms of the disease over a longer period of time. these trials can longer period of time. these trials ca n ofte n longer period of time. these trials ca n often ta ke longer period of time. these trials can often take years. then we want to see if we can slow the disease in people with symptoms and then the next step would be if that is successful, we want to be able to give this drug to people who carry the gene, who are completely well, and 21—day prevent the disease ever occurring, because it is genetic, we have the potential of doing that. occurring, because it is genetic, we have the potential of doing thatm isa have the potential of doing thatm is a fantastic prospect. this disease has been devastating for many thousands of people. if there are people watching who are affected by two, in early stages, what is the message to them in terms of their prospects over the next ten years? this is a massive breakthrough in the development of therapies for this terrible disease, i have been working on huntingdon's disease and have seen patients and families for

20 years and now i feel that there is hope on the horizon and that we will be able to test a potential therapy that one day will be able to slow the disease. i think families and patients, this represents a big step forward. are there any other teams around the world who are working in this area? i am wondering to what extent you really have pioneered this approach, here in the uk with your team, other other parts of the world where people are working on a similar way? there are many people working on molecular therapies for huntingdon's disease and this was important because it started two years ago and was the first trial to try and target disorder therapy. it has been done in five centres in the uk, a centre in canada and three centres in germany and was pioneered with our collea g u es germany and was pioneered with our colleagues at the pharmaceuticals company that developed the drug and we have been helping to take it forward for therapy. it has been a unique collaboration between the us,

the uk and canada and the next trial, we hope to be worldwide. that was the question i was going to ask. only talked about expanding the trial, it will be a global trial. that is the aim. how many people will be involved ? that is the aim. how many people will be involved? you have to calculate how many people you need ona calculate how many people you need on a specific amount of time, it could be several hundred people and probably lasting between one and two yea rs. probably lasting between one and two years. it takes that amount of time to see if you are slowing the disease and slowing the clinical audit symptoms of the disease in patients. i hope you don't mind me asking, i have time to ask you a more political question but i think it is relevant, because you have made this huge advance, i am wondering about the make—up of your team, in terms of backgrounds and nationalities, in this context of brexit, because there has been a controversial debate about to what extent brexit might affect the make—up of teams such as yours and indeed the way you collaborate, you mention germany for example, what would be your answer? the impact of

brexit is going to have a serious impact on the type of research i have described. we collaborate with all countries in europe, the uk leads on drug development and we had the european medicines agency which was critical in getting approvals for drugs in the future, like the one that is in development and i think the most important point for people who are working on brexit now, if they have to make sure that brexit doesn't affect development of innovative therapies for these sorts of diseases, because it has the potential to damage that. how hopeful are you that that will be done, that that will be secured and protected ? done, that that will be secured and protected? i am hoping that the government and everyone who is working on brexit and the european medicines agency and the pharmaceutical companies take that seriously, because for us who are working on essential collaborations with europe, this is a very important subject. they may well be listening to the

interview, wonderful to talk to you and many congratulations again. thank you so much. thousands more people have been told to leave their homes in california, as one of the biggest wildfires in the state's history moves towards the coastal city of santa barbara. around 800 buildings have already been destroyed in the fires that have raged across california last week. firefighters are battling six fires across the state with the largest having destroyed an area of 230,000 acres as our correspondent simon cullen reports. it's a battle being fought both from the ground and from the air. fanned by strong winds and hot weather, fire fighters are struggling to get the upper hand on a blaze which has become one of the most destructive in california's history. hundreds of homes have already been destroyed and thousands of people have been forced to flee.

i'm scared to death. we packed up our whole car. we got evacuated this morning. i'm a nervous wreck. i've only lived in santa barbara five years, so this is the first time i've experienced a fire. so i have been in panic mode all day. the fire in the coastal areas of ventura and santa barbara, has now burnt out an area larger than the size of new york, and it's still out of control. but it's not the only one. and resources are being stretched to the limit. we've been up — i'm at 29 hours straight every other day. everybody on this division is at 28, 29 hours. so we are exhausted. but they are not coming off until this is done. several fire fighters have been injured, and one person, a 70—year—old lady, has died while trying to flee. santa barbara police department! as the flames draw nearer, the mandatory evacuation zone has been widened. overnight, authorities went door

to door, telling people to leave. this fire has acted in an unpredictable manner. it has been very rapidly moving. it's going to be a multi—day event, so please do what you can now to prepare to evacuate. the fire front has become so large, it is clearly visible from space, giving a sense of the magnitude of the task ahead. the us president donald trump has declared a state of emergency to provide extra resources to the worst affected regions. for now, authorities are focusing on bringing the fires under control. but soon, the focus will turn to assessing the damage and counting the cost of the devastating fire season. simon cullen, bbc news. this is bbc news at five — the headlines: disruption continues across the uk in a day which saw more than a thousand schools closed, and hundreds

of homes are without power— all caused by heavy snow. a murder investigation is launched after three children die in a house fire in manchester — a fourth is critically ill in hospital. a man is being held after an attempted terror attack at new york city's main bus terminal left four injured including the bomber. an update on the market numbers for you — here's how london's and frankfurt ended the day. london looking positive and frankfurt making a few losses. and in the the united states this is how the dow and the nasdaq are getting on. quick update on events in the house of commons, boris johnson quick update on events in the house of commons, borisjohnson has been asked about his recent visit to iran and his efforts to secure the release of the dual british citizens

nazanin zaghari—ratcliffe. release of the dual british citizens nazanin zaghari— ratcliffelj release of the dual british citizens nazanin zaghari-ratcliffe. i met the president and the speaker and the foreign minister and had long discussions with the president and in each of those conversations i repeated, as i have said to the house, the case for release on humanitarian grounds, where that is appropriate, and that message was certainly received and understood. i say to the house of commons, it is too early to be confident about the outcome. boris johnson the foreign secretary there in that statement on the house of commons a few moments ago after his recent visit to iran and his attempts to secure the release of nazanin zaghari—ratcliffe. it is 12 minutes to six. keep chegwin has died at the

age of 60 at what his family said was a long—term battle with a progressive lung condition. keith chegwin started his career as an actor and became a household name as actor and became a household name as a television presenter of shows such as swap shop. he really was a fixed feature of children's television and known by millions of children. he died at home with his family by his side and they said that they were heartbroken at his death at the age of 60. noel edmonds who of course worked with keep chegwin on swap shop —— swap shop. he paid tribute to his former colleague. shop —— swap shop. he paid tribute to his former colleaguelj shop —— swap shop. he paid tribute to his former colleague. i had no idea that he was so poorly. it has

come as a profound shock. i am very, very upset at the loss of i think my first tele— charm, we were notjust colleagues, we were good mates. the moving tribute their paid by noel edmonds to cheggers. let us stop to our entertainment correspondent, lots of tributes today, i think we are bound to say, certainly for it those of us who remember him clearly, he is such a familiar performer on all of those big television shows, he was a natural, and natural broadcaster in all senses of the world. he never wrote any scripts, it was about being responsive to the situation. chris evans called him the king of the outside broadcast in his tribute up so many people, they will remember him being on the white cliffs of dover or in scotland, on top of a bridge, trying to explain the situation and that is what he brought to those shows, energy and

enthusiasm. more people aware of the fa ct enthusiasm. more people aware of the fact that he had been battling this long—term condition? fact that he had been battling this long-term condition? it had been getting worse and this year he had not tried to do pantomime which was something he normally did. noel edmonds did not know, they were trying to keep it quite private, a shock to a lot of people. maggie philbin, his first wife paid tribute to him today saying how she had seen him one month ago and it had been so lovely to see him trying to battle, janice long, his sister as well, it is affecting so many people. when you're showing those clips, so many people watching the news channel will be taken back to their childhood, watching those shows, such a huge part, saturday morning tv was enormous when he was honoured and he was honoured for more than a decade. they broke the mould, it was a different style of programming, it was unscripted, exciting, dynamic, it was off the cuff, it is difficult

today to come they know what represented at that time. they had arrival, tiswas on bbc one and tiswas on itv. —— swap shop. just thinking, after the career that we have been describing, we noticed him in different roles, i remember him in different roles, i remember him popping up in a series of extras, where he showed real gift for taking the mickey out of himself, but also a very sharp acerbic sense of humour which was miles away from the kids television performer we have seen previously.

absolutely. we were trying to get a clip suitable for broadcast, there isn't one! it was that route. ricky tulisa used again in a different programme, he appreciated his ability. it is not a career entirely without sadness, he talked about his battle with alcohol in his autobiography but he managed to recover from that and reinvent himself, he was in the big brea kfast. himself, he was in the big breakfast. he does wish he had never done naked jungle. we all get it wrong sometimes! what for you has been the most common theme of the tributes? people have real affection for this guy. fiona phillips said i am so sad to hear that my friend keith chegwin has passed, i will never forget his keith chegwin has passed, i will neverforget his energy, professionalism, kindness or his very bad jokes. a lovely man whose talent was bigger than he realise,

suggesting that he was almost as —— too self—deprecating. gaby roslin, the presenter of the big breakfast, i cannot bear the news, not him, i was blessed to work with keith chegwin, he was a happy, joyous man, so generous and kind, goodbye cheggers. a lot of people will feel that today. thank you for sharing those with us. the uk has a new tallest mountain. mount hope, which is sited in the part of the antarctic claimed by the uk, was recently re—measured and found to tower above what had previously been considered the tallest peak mount jackson, also in the antarctic. our science correspondent jonathan amos reports. you would think the entire world would be mapped to the nearest centimetre by now, but not it seems the antarctic. its rugged, icy terrain is hard to traverse and no one has

yet climbed all its mountains to measure their peaks. cartogrophers rely on satellites to work out the heights of summits. when the british antarctic survey did this recently, they found mount jackson long assumed to be the tallest on the antarctic peninsula was surpassed by mount hope. satellites showed hope to be 3,239 metres high, just over 10,600 feet. the existing believed height of 2,850 metres, goes back to the 1960s from overland survey. now with the availability of sub—metre resolution satellite imagery, we have been able to get an accurate height to plus or minus five metres for mount hope. accurate mapping is vital because scientists fly everywhere on the continent. if mountains aren't featured or are in the wrong place, it can be very dangerous. american researchers are repeating the british re—mapping exercise across the entire continent. it should be finished next year. that will turn antarctica from the worst mapped place

on the planet to one of the best. jonathan amos, bbc news. stand—by for the news that six coming up. time for a look at the weather... here's darren bett. we have had arctic air. we have not seen as much noted a. the greatest depths of snow that we had yesterday we re a cross wales depths of snow that we had yesterday were across wales were as wales today has been sunny and really quite cold. it is the low temperatures we need to concern ourselves with tonight. we had some rain that turned to sleet and snow. that cloud and wet weather has moved through does the cloud has cleared, allowing us to get a peek at the snow cover we have got across many parts of wales. it is with snowfields were we will get the lowest temperatures, still more cloud at least for this evening for

a while in east anglia and the south—east to give us a few more of these wintry showers and a few showers are moving into the western fringes of wales and into cornwall but for many parts of england and wales, it will be clearing skies, lowering temperatures, those wintry showers on these goals all the way into scotland where we have some snow on the hills of scotland, not as much noted a for northern ireland, most of those showers will be rain and as we run through the night, the showers will fade away but with all the lines no, very quickly it could turn very treacherous out there overnight. into tomorrow, as well. getting cold and we will find those showers fading away from northern ireland, from scotland, a few more towards the south west, most of the wintry showers on the north sea coast but for many learned, it is going to be clear skies and it will be a cold night in the south—east than it was last night and our headline temperatures, probably —12 —13 degrees in the countryside over those fields of snow. it will be a

cold start, very cool started tomorrow, potentially an icy one as well, we will soon see the back of the showers in the south west and the showers in the south west and the winds will blow those showers away from these clothes but we will see cloud increasing for the western countries, wind picking up to bring rain in northern ireland, western parts of scotland, but ahead of it it will be a cold day after that widespread frost, but a dry day with light winds and plenty of sunshine. that weather front coming into the cold air means the risk of snow in the scottish hills, the hills of the north of england, the threat of icy conditions as well before we blow in some atlantic winds, some westerly winds, a change for a wednesday, more cloud around, sherry bursts of rain pushes into the east as we get some late sunshine in scotland, the airwill turn some late sunshine in scotland, the air will turn colder, we will see some wintry showers around as well. nothing too serious, nothing like as cold as we saw yesterday nor as we are seeing tonight. tonight at six: snow gets the better of britain — a catalogue of disruption across the country. drivers are being told to take care, flights have been cancelled

and there are delays on the railways. a big job to get things right again. we've covered something like 17,000 miles of gritting, which is from gloucester to australia and back, trying to keep the place moving. a day of fun on the snow as more than 1,000 schools in england and wales shut their gates. and there are warnings of treacherous conditions as snow turns to ice for many tonight. also on the programme: a breakthrough that brings hope for patients with huntington's disease — the biggest advance for 50 years. manchester police launch a murder investigation after three children die in a suspicious house fire. an attempted terror attack at new york's main bus station — the bomber and some bystanders are injured.