Recessive dystrophic epidermolysis bullosa keratinocytes synthesize type VII collagen alpha chains of normal molecular size but the anchoring fibril assembly is impaired.


Recessive dystrophic epidermolysis bullosa is a blistering skin disease characterized by diminished anchoring fibrils within the cutaneous basement membrane zone. Because anchoring fibrils are composed of type VII collagen, we compared the synthesis of type VII collagen by keratinocytes from patients and normal subjects. By Western blot analysis and… (More)


  • Presentations referencing similar topics