anchoring fibrils
{{Nofootnotes|article|date=December 2013}}
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.{{cite journal |doi= 10.1083/jcb.111.5.2109 |title= Epithelial origin of cutaneous anchoring fibrils |year= 1990 |last1= Regauer |first1= S. |journal= The Journal of Cell Biology |volume= 111 |issue= 5 |pages= 2109–15 |pmid= 2229187 |last2= Seiler |first2= GR |last3= Barrandon |first3= Y |last4= Easley |first4= KW |last5= Compton |first5= CC |pmc= 2116344}}
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.{{Cite journal|last1=Bardhan|first1=Ajoy|last2=Bruckner-Tuderman|first2=Leena|last3=Chapple|first3=Iain L. C.|last4=Fine|first4=Jo-David|last5=Harper|first5=Natasha|last6=Has|first6=Cristina|last7=Magin|first7=Thomas M.|last8=Marinkovich|first8=M. Peter|last9=Marshall|first9=John F.|last10=McGrath|first10=John A.|last11=Mellerio|first11=Jemima E.|date=2020-09-24|title=Epidermolysis bullosa|url=https://www.nature.com/articles/s41572-020-0210-0|journal=Nature Reviews Disease Primers|language=en|volume=6|issue=1|page=78|doi=10.1038/s41572-020-0210-0|pmid=32973163|s2cid=221861310|issn=2056-676X}}{{cite journal |doi=10.1111/1523-1747.ep12365129 |title=Type VII Collagen, Anchoring Fibrils, and Epidermolysis Bullosa |year=1993 |last1=Burgeson |first1=Robert E. |journal=Journal of Investigative Dermatology |volume=101 |issue=3 |pages=252–5 |pmid=8370960|doi-access= }} 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.{{cite journal |doi=10.1016/S0945-053X(98)00007-9 |title=Biology of anchoring fibrils: Lessons from dystrophic epidermolysis bullosa |year=1999 |last1=Bruckner-Tuderman |first1=Leena |last2=Höpfner |first2=Bianca |last3=Hammami-Hauasli |first3=Nadja |journal=Matrix Biology |volume=18 |pages=43–54 |pmid=10367730 |issue=1}}
See also
References
{{Reflist}}
- {{cite journal |first1=Douglas R. |last1=Keene |first2=Lynn Y. |last2=Sakai |first3=Gregory P. |last3=Lunstrum |first4=Nicholas P. |last4=Morris |first5=Robert E. |last5=Burgeson |pmid=3818794 |doi=10.1083/jcb.104.3.611 |year=1987 |title=Type VII collagen forms an extended network of anchoring fibrils |volume=104 |issue=3 |pages=611–21 |pmc=2114566 |journal=The Journal of Cell Biology}}
- {{cite journal |doi=10.1111/1523-1747.ep12471830 |title=Structural Variations in Anchoring Fibrils in Dystrophic Epidermolysis Bullosa: Correlation with Type VII Collagen Expression |year=1993 |last1=McGrath |first1=John A. |last2=Ishida-Yamamoto |first2=Akemi |last3=O'Grady |first3=Anthony |last4=Leigh |first4=Irene M. |last5=Eady |first5=Robin A. J. |journal=Journal of Investigative Dermatology |volume=100 |issue=4 |pages=366–72 |pmid=8454899|doi-access= }}
External links
- {{cite web |url=http://www.thedoctorsdoctor.com/bodysites/skin_bm.htm |title=Skin Basement Membrane |date=April 10, 2006 |publisher=The Doctor's Doctor}}
{{Connective tissue}}