期刊
DERMATOLOGIC CLINICS
卷 28, 期 1, 页码 93-+出版社
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1016/j.det.2009.10.011
关键词
Epidermolysis bullosa; Type VII collagen; Anchoring fibrils; Heritable blistering diseases
类别
资金
- DHHS
- NIH/NIAMS [P01 AR38923, R01 AR54876]
- Dermatology Foundation
- NATIONAL INSTITUTE OF ARTHRITIS AND MUSCULOSKELETAL AND SKIN DISEASES [R01AR054876, P01AR038923] Funding Source: NIH RePORTER
Type VII collagen is a major component of the anchoring fibrils of the dermal-epidermal adhesion on the dermal side at the lamina densa/papillary dermis interface. Dystrophic epidermolysis bullosa (DEB) emerged as a candidate for type VII collagen mutations becausing anchoring fibrils were shown to be morphologically altered, reduced in number, or completely absent in patients with different forms of DEB. Circulating autoantibodies recognize type VII collagen epitopes in epidermolysis bullosa acquisita. The suggestion that type VII collagen is required for human epidermal tumorigenesis relates to the increasing numbers of life-threatening complications associated with developing squamous cell carcinomas because of the extended life span of affected individuals with recessive DEB.
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