BP180/Collagen XVII: A Molecular View

BP180 is a type II collagenous transmembrane protein and is best known as the major autoantigen in the blistering skin disease bullous pemphigoid (BP). The BP180 trimer is a central component in type I hemidesmosomes (HD), which cause the adhesion between epidermal keratinocytes and the basal lamina...

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Main Authors: Jussi Tuusa, Nina Kokkonen, Kaisa Tasanen
Format: Article
Language:English
Published: MDPI AG 2021-11-01
Series:International Journal of Molecular Sciences
Subjects:
Online Access:https://www.mdpi.com/1422-0067/22/22/12233
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author Jussi Tuusa
Nina Kokkonen
Kaisa Tasanen
author_facet Jussi Tuusa
Nina Kokkonen
Kaisa Tasanen
author_sort Jussi Tuusa
collection DOAJ
description BP180 is a type II collagenous transmembrane protein and is best known as the major autoantigen in the blistering skin disease bullous pemphigoid (BP). The BP180 trimer is a central component in type I hemidesmosomes (HD), which cause the adhesion between epidermal keratinocytes and the basal lamina, but BP180 is also expressed in several non-HD locations, where its functions are poorly characterized. The immunological roles of intact and proteolytically processed BP180, relevant in BP, have been subject to intensive research, but novel functions in cell proliferation, differentiation, and aging have also recently been described. To better understand the multiple physiological functions of BP180, the focus should return to the protein itself. Here, we comprehensively review the properties of the BP180 molecule, present new data on the biochemical features of its intracellular domain, and discuss their significance with regard to BP180 folding and protein–protein interactions.
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spelling doaj.art-a712ee00d3df459ab190b6be5b095e4b2023-11-22T23:39:09ZengMDPI AGInternational Journal of Molecular Sciences1661-65961422-00672021-11-0122221223310.3390/ijms222212233BP180/Collagen XVII: A Molecular ViewJussi Tuusa0Nina Kokkonen1Kaisa Tasanen2PEDEGO Research Unit, Department of Dermatology, Medical Research Center Oulu, Oulu University Hospital and University of Oulu, P.O. Box 8000, FI-90014 Oulu, FinlandPEDEGO Research Unit, Department of Dermatology, Medical Research Center Oulu, Oulu University Hospital and University of Oulu, P.O. Box 8000, FI-90014 Oulu, FinlandPEDEGO Research Unit, Department of Dermatology, Medical Research Center Oulu, Oulu University Hospital and University of Oulu, P.O. Box 8000, FI-90014 Oulu, FinlandBP180 is a type II collagenous transmembrane protein and is best known as the major autoantigen in the blistering skin disease bullous pemphigoid (BP). The BP180 trimer is a central component in type I hemidesmosomes (HD), which cause the adhesion between epidermal keratinocytes and the basal lamina, but BP180 is also expressed in several non-HD locations, where its functions are poorly characterized. The immunological roles of intact and proteolytically processed BP180, relevant in BP, have been subject to intensive research, but novel functions in cell proliferation, differentiation, and aging have also recently been described. To better understand the multiple physiological functions of BP180, the focus should return to the protein itself. Here, we comprehensively review the properties of the BP180 molecule, present new data on the biochemical features of its intracellular domain, and discuss their significance with regard to BP180 folding and protein–protein interactions.https://www.mdpi.com/1422-0067/22/22/12233autoimmune diseasebullous pemphigoidhemidesmosomeintrinsically disorderedkeratinocyteprotein–protein interaction
spellingShingle Jussi Tuusa
Nina Kokkonen
Kaisa Tasanen
BP180/Collagen XVII: A Molecular View
International Journal of Molecular Sciences
autoimmune disease
bullous pemphigoid
hemidesmosome
intrinsically disordered
keratinocyte
protein–protein interaction
title BP180/Collagen XVII: A Molecular View
title_full BP180/Collagen XVII: A Molecular View
title_fullStr BP180/Collagen XVII: A Molecular View
title_full_unstemmed BP180/Collagen XVII: A Molecular View
title_short BP180/Collagen XVII: A Molecular View
title_sort bp180 collagen xvii a molecular view
topic autoimmune disease
bullous pemphigoid
hemidesmosome
intrinsically disordered
keratinocyte
protein–protein interaction
url https://www.mdpi.com/1422-0067/22/22/12233
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AT ninakokkonen bp180collagenxviiamolecularview
AT kaisatasanen bp180collagenxviiamolecularview