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takyon [soylentnews.org] writes:

'Butterfly child' given life-saving skin [bbc.com]

A child has been given a new genetically modified skin that covers 80% of his body, in a series of lifesaving operations. Hassan, who lives in Germany, has a genetic disease - junctional epidermolysis bullosa - that leaves his skin as fragile as a butterfly's wings. A piece of his skin was taken, its DNA was repaired in the laboratory and the modified skin grafted back on. After nearly two years, the new skin appears completely normal.

[...] Normally, the different layers of the skin are held together by "anchoring proteins". But the junctional epidermolysis bullosa means Hassan's DNA lacks the instructions for sticking his epidermis (the surface layer) to the dermis (the next one down). There is no cure, and about four in 10 patients do not even reach adolescence.

[...] [A] team of biologists specialising in gene therapy were brought in from the University of Modena and Reggio Emilia, in Italy - and the parents gave approval for them to try an experimental therapy.

In September 2015, a 4 sq cm (0.6 sq inches) patch of skin was taken from an area where the epidermis was still intact. The biopsy was then infected with a customised virus. Viruses are good at getting inside cells, and this one contained the missing instructions for binding the layers of skin together.

The now genetically modified skin cells were grown to make skin grafts totalling 0.85 sq m (9 sq ft). It took three operations over that winter to cover 80% of the child's body in the new skin. Hassan's father said his son had spent months covered in so many bandages he had looked like a mummy. But 21 months later, the skin is functioning normally with no sign of blistering. You can even pinch the once incredibly fragile skin, with no sign of damage.

[...] An analysis of the structure of Hassan's skin, detailed in the journal Nature [nature.com] [DOI: 10.1038/nature24487] [DX [doi.org]], has discovered a group of long-lived stem cells are that constantly renewing his genetically modified skin.

Also at NYT [nytimes.com] and The Washington Post [washingtonpost.com] (archive [archive.is]).

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