02/08/2017 World News Today


02/08/2017

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Scientists think they are a step closer to preventing inherited

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diseases being passed on in families.

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They've found a way to edit genes, removing the faulty DNA

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A method of being able to avoid having an effect on children,

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passing on the defective gene, could be really very important

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for those families and allow them to get out of this.

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"Tampered with" - the company providing the technology

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for Venezeula's controversial election for a new assembly says

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Worth a quarter of a billion dollars - if Neymar moves

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to Paris Saint-Germain it would be the world's biggest

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And the end of an era - Prince Philip carries out his last

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scheduled public engagement and retires at the age of 96.

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Hello and welcome to World News Today.

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For the first time scientists have successfully repaired a faulty

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They used a process known as "gene editing" to correct DNA that causes

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The US and South Korean team allowed the embryos to develop for five days

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The technique is still at the early research stage but it raises

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the hope of preventing 10,000 gene disorders which pass

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Our medical correspondent Fergus Walsh reports.

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The goal could not be more ambitious, to eradicate

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These scientists have taken an impressive first

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step on a long road, editing DNA in human embryos.

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Inside the nucleus of each of our cells is our genome,

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It is the instruction manual for life.

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The scientists were targeting a faulty gene that causes

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They fertilise the egg with the sperm of it a man who carried the

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faulty gene. They then injected the gene editing

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system, which scans the DNA It then cuts both strands

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of the DNA, and removes the faulty gene, a healthy copy of the gene

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from the egg is then Here are some of the embryos

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from the study in the journal Nature after being edited,

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42 of 58 embryos were corrected. They were allowed to develop

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for five days, none was implanted. The research has been welcomed

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by a team in London, who have a license

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to edit human embryos. They say the technology

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could eventually help many families. There are some nasty genetic

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diseases like Huntingdon's, or a disease that affects heart

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function later in life, which can blight families

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for many generations. So a method of being able to avoid

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having affected children, passing on the defective gene,

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can be really very important Nicole Mowbray has the same

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heart condition that was She now has a defibrillator

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implanted in her chest, She has a 50% risk of passing

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on the condition, but is unsure of whether she would

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consider gene editing. I wouldn't want to pass on something

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that caused my child to have a limited life or a painful

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life, or a life of risk. That does, obviously,

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come to the front of my mind I wouldn't want to create

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the perfect child, in inverted commas, I feel like my condition

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makes me me. Previous attempts at editing human

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embryos in China led There is a lot of work needed before

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this can be considered safe, and it raises ethical issues

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about how far science should go In a deal that could be worth more

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than a quarter of a billion - yes, billion - dollars,

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the Brazilian football player Neymar appears to be a step closer

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to leaving Barcelona and signing That figure is more than twice

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the current world record transfer Our correpsondent Richard

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Conway is in Paris. Fans visiting the club shop

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here in the centre of Paris are getting excited because the deal

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for Neymar is inching We have seen in the past few days

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the player going from Shanghai where he was on commercial duties

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and flying back to Barcelona. He has been excused from training

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and now Barcelona are saying, pay us the money and Paris St Germain,

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you can have the player. We will see how it plays out

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in the coming hours and days. There is now an increasing

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certainty that Neymar will be a Paris Saint-Germain player this

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coming season and the indications for that are huge, of course

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for Barcelona, who will be without one of their leading players

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and for PSG who desperately want to bridge the gap

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from being Champions League wannabes to winners and also for Uefa,

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given Financial Fair Play, clubs have a requirement

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to live within their means. The size of this deal and the money

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involved in this potential transfer is such that many clubs

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and individuals at the highest levels of football are looking at it

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and wondering if PSG can live For now the fans here just

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want to know one thing, With me is the football

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journalist Tim Vickery. I read your article or later. Is

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this a negotiating ploy or does look like a deal? It looks like it is

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going to happen. The speculation and negotiation comes as no surprise

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whatsoever. I am still a little surprised that it looks like it is

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Neymar's decision. He wants to go. I just hope it is his decision and

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what he wants to do. Not what his entourage would prefer. It is not

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quite as simple as signing Neymar, is it? He has a complicated

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structure around him. Including his father. You always worries about

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that because it condemns the footballer to a permanent

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childishness, if you like. But his career has always been managed by

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his father on a step-by-step basis. Amongst the final objectives is

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winning the Fever world Player of the Year award. That is taken with

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great importance in Brazil. Five Brazilians won on it different

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occasions. Neymar will be seen by himself and many of his compatriots

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as something of a disappointment that he does not win that a word. He

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is unlikely to win it if he is not outstanding player in his own team.

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And also if he does not have a team around him. People from his camp are

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saying he has been seduced by this project of Paris Saint-Germain. And

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it will be the World Cup sin, where he plays at 26 years old, closest to

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his peak, and perhaps during the French lead they will be able to

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coast a little more leaving him with enough gas in the tank for the World

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Cup next year. And still able to walk away from the experience of

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playing up front with Luis Suarez and Lionel Messi. The people in his

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entourage with the strategy do not have the joy he has, like a

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collective childhood they have on a weekly basis with his players, there

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is no animosity between them, they look like one force, and it is sad

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in one way that he is turning his back on that and you just hope it

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really is what he wants to do. And you can find that article on the BBC

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sport website. Venezuela's President Nicolas Maduro

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plans to swear in a new assembly It follows a controversial election

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in the country on Sunday, where the turn-out figure

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was manipulated - that's according to the technology company

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which provided the voting system. Antonio Mugica, the boss

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of Smartmatic, said his firm estimated the difference

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between actual participation and the one announced

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by the authorities was at least It is therefore with the deepest

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regret that we have to report that the turnout numbers on Sunday

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30th of July for the constituent assembly in Venezuela

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were tampered with. The automated election system used

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in the Venezuela is tamper-evident and self-reports any attempts

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to interfere with it. Our correspondent Will Grant

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is in Caracas for us now. Has the government responded? Yes,

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they are currently issuing the credentials for the new deputies in

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quite a ceremony outside the building of the National electoral

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Council and every high-profile new deputy, including Nicholas Monroe's

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wife -- Maduro's wife. There does not seem to be any recognition of

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the comment about the tampered votes from them. Reuters started the day

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saying they had seen an internal memo from the Venezuelan electoral

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authorities that suggested by half past five on the day of voting only

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3.7 million people had voted, and the polls were due to close a couple

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of hours later, and the government said 8 million people voted, so how

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something around 4.3 or 4.4 million people voted in just a couple of

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hours is beyond most people's imagination in Venezuela. It strikes

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me that almost everything in Venezuelan society has become

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politicised. Can we say that this firm making these claims is neutral?

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That is a very good question. The thing is, I think, that is

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interesting and important here is that the government have heralded

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that from themselves as the reason the elections are free and fair.

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They have done the elections and run the voting machines here since 2004,

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so the idea that they suddenly wouldn't they, and they are all very

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well respected company, used around the world for elections everywhere

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from the Philippines to parts of Latin America, so you're right,

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everything in Venezuela is politicised and no doubt this will

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be too but the Maduro government has always pointed to this company and

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their machines as the reason why results are believable.

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Let's take a look at some of the other stories making the news.

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A man and a child have been killed after a small aircraft made

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an emergency landing on a beach in Portugal.

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Local media reports say the incident took place on a beach

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at Caparica in Almada, south west of Lisbon.

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Reports said the pair were sunbathing when the plane

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A local newspaper said members of the plane's crew were unharmed

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and were being interviewed by authorities.

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The US Secretary of State, Rex Tillerson, has stressed

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that his country is not seeking to topple the North Korean

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government and it wants dialogue with Pyongyang.

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Mr Tillerson said, "We are not your enemy",

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but warned Pyongyang that its ballistic missile

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tests were presenting an "unacceptable threat" to the US,

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and Washington would have to respond.

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The boss of Apple has said his company had no choice

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but to stop providing apps in China that allow users to circumvent

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the country's tough internet censorship.

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Tim Cook said the technology firm would rather not have stopped

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offering virtual private network, or VPN apps, but had

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Amazon has also confirmed that customers using its cloud

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computing services cannot now use unauthorised VPNs.

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And we just want to show you one marine researcher's close encounter

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Definitely not safe to go back in the water.

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Well, luckily it was only a marine researcher's camera the shark

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Greg Skomal was diving off the coast of Massachusetts in the US.

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Both he and his camera remained intact.

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Scientists have used a process called gene editing to correct DNA

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that causes a deadly heart condition. Fergus Walsh had the

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story and I played his report earlier. How easily do the

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techniques translate more broadly to genetic issues? This particular form

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of gene editing, it was only discovered five years ago and it is

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incredibly cheap and easy and efficient so it has been adopted by

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medical researchers, animal and plant researchers, because you can

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edit the DNA of any living object using this technique. The sky is the

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limit regarding how it can be used. In terms of 10,000 inherited gene

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disorders there is enormous potential. But we are really at the

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foothills of a giant mountain that needs to be claimed in terms of

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safety, efficiency. Some Chinese researchers had a previous call at

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this. Embryos they used were not very good quality, they were not

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clinical grade, and they had all sorts of errors and the one thing

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you do not want to do is to do this in the clinic with couples wanting

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to have a healthy child, a risk of passing on a fatal heart disorder,

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and then find you have created another problem. So it is a long way

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before we will see this in the clinic. Who decides the ethics of

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this? Individual countries. There was a conference in Washington 18

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months ago that I attended and it called for research to continue but

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a moratorium on using human embryo gene editing in the clinic. There

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are observers who are unhappy that this team from the US and South

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Korea have done this work. They said maybe they should be sticking to

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check in on mouse embryos -- chicken or mouse embryos. One country

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regarded internationally and sending good signals in terms of keeping an

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eye on what is happening is the UK. A team in London has also been given

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permission to do gene editing in human embryos and that has gone

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through the regulator here and they will be publishing their research at

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some point. They are also doing chicken and mouse embryos. The

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concern is that when something is possible, and it really is very

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easy, I have seen people do this gene editing in their garage in

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California when I was doing a documentary on it. When something is

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possible people will just do it. But then we had the same fears about

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human cloning many years ago as well. If you want more information

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on this story you can get it on the BBC news at.

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Thousands of Syrian refugees and fighters are being

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evacuated from camps on the Lebanese border today.

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The operation is part of a ceasefire deal struck

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between fighters belonging to the Lebanese Shia Hezbollah

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Over 100 buses will transport Syrian civillians, and also jihadi

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fighters, back into Syria and our Correspondent Carine Torbey

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is on the Lebanese border with the latest.

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This is the road that around 150 buses carrying thousands

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of civilians and hundreds of fighters from Jabhat Fateh

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al-Sham, the previously known al-Nusra Front,

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will be taking out of here, the outskirts of Arsal,

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on the border between Lebanon and Syria, into Syria.

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The convoy of buses will be accompanied by fighters

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from Hezbollah who drove the militants out of these

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mountains just two weeks ago in a six-day battle.

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The journey between here and Syria is expected to be a long

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and exhausting one and once the buses arrive to the opposition

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held area of Idlib, five Hezbollah fighters who were held hostage

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The completion of this whole operation will mark the end

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of the presence of militants from what was previously known

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as al-Nusra Front in Lebanon and is expected to further secure

:19:05.:19:09.

Poland is refusing to comply with a European Court order to stop

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logging in Europe's last remaining tract of primeval forrest.

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The government argues it's necessary to control a beetle outbreak.

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If the dispute remains unresolved, it could lead to hefty financial

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penalties and further strain the already tense relationship

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A relic of ancient woodlands and home to one of the world's

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largest population of European bison.

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Covering almost 150,000 hectares on the border of Poland and Belarus,

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the Unesco-listed Bialowieza forest is also the focus of a legal

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tussle over logging that has gone all the way

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It has issued a preliminary decision ordering the immediate

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suspension of all logging, but Poland is refusing

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to comply with the order, arguing it is trying to contain

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TRANSLATION: We have to fulfil the protective measures planned

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for the forest and this is what we are doing.

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The Polish government says authorities are conducting

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an experiment, which could last several years, to work out the best

:20:24.:20:26.

By then, though, conservationists warn the logging will have

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The dispute has led to scuffles on the ground.

:20:34.:20:38.

A cameraman was allegedly assaulted by a timber worker while trying

:20:39.:20:41.

to film logging operations after the court order

:20:42.:20:43.

The legal stand-off is just the latest challenge by Poland

:20:44.:20:50.

to the European Union's authority and could lead to a 4 million euros

:20:51.:20:53.

fine along with daily penalties if it continues unresolved.

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After 65 years of official public engagements, Britain's Prince Philip

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has made his final solo appearance on royal duty today.

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He attended a parade by the Royal Marines,

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Now 96, he's the longest serving consort in British history.

:21:14.:21:19.

Our Royal Correspondent Nicholas Witchell is at

:21:20.:21:21.

It was the kind of afternoon weather-wise which might have made

:21:22.:21:32.

Quite apart from the fact that in the Duke's case he's been doing

:21:33.:21:36.

But there he was, on the forecourt of Buckingham Palace, a man of 96,

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standing to attention in the pouring rain for the salute he has

:21:42.:21:44.

There were many things to remind him of the past decade.

:21:45.:21:53.

The parade had been mounted by the Royal Marines,

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the fighting force which is part of his beloved Royal Navy.

:21:56.:21:58.

And in the background was the Palace where he has

:21:59.:22:04.

attended so many events, garden parties and the formal

:22:05.:22:06.

And where his programme, 22,000 solo engagements,

:22:07.:22:12.

more than 5,000 speeches, has been planned.

:22:13.:22:18.

The Duke strode across the forecourt, no stick for him

:22:19.:22:22.

and woe betide anyone who might suggest such a thing.

:22:23.:22:24.

And as he went, the crowd outside the Palace applauded.

:22:25.:22:27.

The Royal Marines gave him three cheers.

:22:28.:22:38.

The Duke waved his hat and strode away.

:22:39.:22:42.

And as he went the band of Her Majesty's Royal Marines

:22:43.:22:47.

After 70 years' service, and with his own separate programme

:22:48.:23:01.

of royal engagements now concluded, who today would have

:23:02.:23:03.

Nicholas Witchell, BBC News, Buckingham Palace.

:23:04.:23:18.

President Donald Trump has called the Russia sanctions legislation

:23:19.:23:20.

he just signed into law, quote, "significantly flawed".

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The new legislation is in response to alleged Russian interfering

:23:23.:23:24.

in the US election and limits Mr Trump's ability

:23:25.:23:26.

to negotiate sanctions without Congressional approval.

:23:27.:23:34.

For more, I'm joined by our correspondent

:23:35.:23:35.

If he doesn't like it, why did he sign it? He was in a corner.

:23:36.:23:50.

Congress went through with the legislation, unanimously approved by

:23:51.:23:53.

Republicans and Democrats, and the way that American democracy works as

:23:54.:24:02.

it landed on his desk. It sat there all weekend, he said he would sign

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it and he has but he has issued a flurry of responses as the number of

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reasons why he does not like it. One of those is that it will limit his

:24:10.:24:17.

ability to lift or waive sanctions against Russia or any other country

:24:18.:24:21.

in future but it also places limits on the amount of money that

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Americans can invest in, for instance, Russian energy projects.

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He believes some of these regulations will limit the ability

:24:30.:24:32.

for American firms to do business in Russia. That is one reason why he

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says he is finding this significantly flawed. But he does

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feel that Congress is stepping on his toes, overreaching itself,

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another reason why he says this is unconstitutional. Has Congress

:24:49.:24:54.

restricted presidents in a similar way in the past? They have done in

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the past but this is quite an unusual move by Congress and by

:25:01.:25:04.

signing it Donald Trump is effectively making it law which

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means he has to abide by it. He did try to work with Congress to try to

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change the language of the bill but here we are, he has been boxed into

:25:17.:25:21.

a corner, and Russia already retaliated over the weekend by

:25:22.:25:27.

saying they would expel half of the diplomatic staff, US diplomatic

:25:28.:25:31.

staff working in Russia. His hope of a new reset with Russia is in

:25:32.:25:34.

tatters at the moment. Our top story, for the first time

:25:35.:25:46.

scientists have prepared a faulty gene in human embryos using a

:25:47.:25:54.

process known as gene editing to correct DNA against a deadly heart

:25:55.:26:02.

Today was a pretty miserable day for large parts of the United Kingdom

:26:03.:26:14.

and it remains pretty unsettled over the next few

:26:15.:26:16.

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