Biology:Anchoring fibrils
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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.[1] This causes a weak dermoepidermal junction, where the epidermis easily separates from the dermis causing much pain. This condition is caused by a mutation of COL7A1, the gene that codes for a type of collagen 7.
See also
- Epidermis (skin)
- Dermis
- Lamina propria
- Connective tissue
References
- ↑ Bardhan, Ajoy; Bruckner-Tuderman, Leena; Chapple, Iain L. C.; Fine, Jo-David; Harper, Natasha; Has, Cristina; Magin, Thomas M.; Marinkovich, M. Peter et al. (2020-09-24). "Epidermolysis bullosa" (in en). Nature Reviews Disease Primers 6 (1): 78. doi:10.1038/s41572-020-0210-0. ISSN 2056-676X. PMID 32973163. https://www.nature.com/articles/s41572-020-0210-0.
- Regauer, S.; Seiler, GR; Barrandon, Y; Easley, KW; Compton, CC (1990). "Epithelial origin of cutaneous anchoring fibrils". The Journal of Cell Biology 111 (5): 2109–15. doi:10.1083/jcb.111.5.2109. PMID 2229187.
- Keene, Douglas R.; Sakai, Lynn Y.; Lunstrum, Gregory P.; Morris, Nicholas P.; Burgeson, Robert E. (1987). "Type VII collagen forms an extended network of anchoring fibrils". The Journal of Cell Biology 104 (3): 611–21. doi:10.1083/jcb.104.3.611. PMID 3818794.
- Bruckner-Tuderman, Leena; Höpfner, Bianca; Hammami-Hauasli, Nadja (1999). "Biology of anchoring fibrils: Lessons from dystrophic epidermolysis bullosa". Matrix Biology 18 (1): 43–54. doi:10.1016/S0945-053X(98)00007-9. PMID 10367730.
- Burgeson, Robert E. (1993). "Type VII Collagen, Anchoring Fibrils, and Epidermolysis Bullosa". Journal of Investigative Dermatology 101 (3): 252–5. doi:10.1111/1523-1747.ep12365129. PMID 8370960.
- McGrath, John A.; Ishida-Yamamoto, Akemi; O'Grady, Anthony; Leigh, Irene M.; Eady, Robin A. J. (1993). "Structural Variations in Anchoring Fibrils in Dystrophic Epidermolysis Bullosa: Correlation with Type VII Collagen Expression". Journal of Investigative Dermatology 100 (4): 366–72. doi:10.1111/1523-1747.ep12471830. PMID 8454899.
External links
- "Skin Basement Membrane". The Doctor's Doctor. April 10, 2006. http://www.thedoctorsdoctor.com/bodysites/skin_bm.htm.
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