Collagen VII deficient mice show morphologic and histologic corneal changes that phenotypically mimic human dystrophic epidermolysis bullosa of the eye.

Experimental Eye Research(2018)

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摘要
•Epidermolysis bullosa (EB) is a devastating disease that causes severe eye pain and vision loss, there are no treatments.•Therapies for EB skin disease are in phase 1–2 pharmaceutical development but cannot be used for eye disease without animal testing.•There are no published articles that describe the eyes of mice with EB.•This mouse model shows multiple biomarkers for fibrosis.•This study provides evidence that hypomorphic mice mimic human disease and are appropriate for testing therapeutics.
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关键词
Collagen VII,Epidermolysis bullosa,DEB,RDEB,Cornea,Eye,Scarring,Opacity,Vision loss,Blindness,Mouse,Murine
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