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a very slender natural or synthetic fiber (同)filament, strand

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微小繊維 / (植物の)根毛 / (生物体の組織の)原線維

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出典(authority):フリー百科事典『ウィキペディア（Wikipedia）』「2016/07/12 08:58:21」(JST)

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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. 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.

References

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. PMC 2116344. 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. PMC 2114566. 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.

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English Journal

High Local Concentrations of Intradermal MSCs Restore Skin Integrity and Facilitate Wound Healing in Dystrophic Epidermolysis Bullosa.

Kühl T1, Mezger M2, Hausser I3, Handgretinger R2, Bruckner-Tuderman L1, Nyström A1.
Molecular therapy : the journal of the American Society of Gene Therapy.Mol Ther.2015 Aug;23(8):1368-79. doi: 10.1038/mt.2015.58. Epub 2015 Apr 10.
Dystrophic epidermolysis bullosa (DEB) is an incurable skin fragility disorder caused by mutations in the COL7A1 gene, coding for the anchoring fibril protein collagen VII (C7). Life-long mechanosensitivity of skin and mucosal surfaces is associated with large body surface erosions, chronic wounds,
PMID 25858020

Dystrophic epidermolysis bullosa: a review.

Shinkuma S1.
Clinical, cosmetic and investigational dermatology.Clin Cosmet Investig Dermatol.2015 May 26;8:275-84. doi: 10.2147/CCID.S54681. eCollection 2015.
Dystrophic epidermolysis bullosa is a rare inherited blistering disorder caused by mutations in the COL7A1 gene encoding type VII collagen. The deficiency and/or dysfunction of type VII collagen leads to subepidermal blistering immediately below the lamina densa, resulting in mucocutaneous fragility
PMID 26064063

Making more matrix: enhancing the deposition of dermal-epidermal junction components in vitro and accelerating organotypic skin culture development, using macromolecular crowding.

Benny P1, Badowski C, Lane EB, Raghunath M.
Tissue engineering. Part A.Tissue Eng Part A.2015 Jan;21(1-2):183-92. doi: 10.1089/ten.TEA.2013.0784. Epub 2014 Oct 9.
Skin is one of the most accessible tissues for experimental biomedical sciences, and cultured skin cells represent one of the longest-running clinical applications of stem cell therapy. However, culture-generated skin mimetic multicellular structures are still limited in their application by the tim
PMID 25058150

Japanese Journal

電顕トモグラフィーによる結合組織線維の3D観察

大澤得二,石田欣二,遠山稿二郎
医学生物学電子顕微鏡技術学会誌 = Journal of electron microscopy technology for medicine and biology 25(1), 20-22, 2011
NAID 40019494304

Pretibial dystrophic epidermolysis bullosa with localized cutaneous amyloidosis : Coincidental or secondary amyloidosis?

AOKI Mikako,NIIMI Yayoi,ISHIKO Akira,KAWANA Seiji
Journal of dermatology 37(3), 259-263, 2010-03-01
NAID 10027066279

Ultraviolet (UV)- and UV-Related Cytokine-Mediated Transcriptional Mechanisms of Type VII Collagen Gene (COL7A1) Expression in the Skin, with Special Reference to Photoaged Skin and Anti-Aging [Emerging Theories of Host Defense. The 10th Meeting Hirosaki International Forum of Medical Science. Communication Center Hirosaki University School of Medicine. Hirosaki on November 21-22, 2006]

Kon Atsushi,Igarashi Manami,Yamaguchi Masanori [他],Kojima Kaoru
弘前醫學 59(Suppl), S154-S161, 2007
… Chronic ultraviolet （UV） radiation results in photoaged skin characterized by deep wrinkle formation.Recent studies suggest that diminishment of anchoring fi brils（ AF）, stabilizing the association of the basement membraneto the underlying dermis, are involved in photoaged skin. …
NAID 110006602866