Anchoring fibrils
Anchoring fibrils (composed largely of type VII collagen) extend from the basal lamina of epithelial cells and attach to the lamina reticularis (also known as the reticular lamina) by wrapping around the reticular fiber (collagen III) bundles. The basal lamina and lamina reticularis together make up the basement membrane. Anchoring fibrils are essential to the functional integrity of the dermoepidermal junction.
Epidermolysis bullosa dystrophica
Epidermolysis bullosa dystrophica, also known as Dystrophic EB (DEB) is a chronic skin condition caused when anchoring fibrils are abnormal, diminished, or absent. This causes a weak dermoepidermal junction, where the epidermis easily separates from the dermis causing much pain. This condition is caused by a genetic defect coding for anchoring fibrils.
See also
References
- Sigrid Regauer, Gregory R. Seiler, Yann Barrandon, Karen W. Easley, Carolyn C. Compton. "Epithelial origin of cutaneous anchoring fibrils." J Cell Biol. 1990 November 1; 111(5): 2109–2115.
- Douglas R. Keene, Lynn Y. Sakai, Gregory P. Lunstrum, Nicholas P. Morris, Robert E. Burgeson. "Type VII collagen forms an extended network of anchoring fibrils." J Cell Biol. 1987 March 1; 104(3): 611–621.
- Bruckner-Tuderman L. "Biology of anchoring fibrils: lessons from dystrophic epidermolysis bullosa." Matrix Biology. 1999 February 1; 18(1): 43-54.
- Burgeson, R. E. "Type VII collagen, anchoring fibrils, and epidermolysis bullosa." Journal of investigative dermatology. 1993 October 28; 101(3): 252-255. Link
- McGrath J. A., Ishida-Yamamoto A., O'Grady A., Leigh I. M., Eady R. A. J. "Structural variations in anchoring fibrils in dystrophic epidermolysis bullosa: correlation with type VII collagen expression." Journal of investigative dermatology. 1993 March 4; 100(4): 366-372. Link
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