BP180/Collagen XVII: A Molecular View

• Published in: International journal of molecular sciences Vol. 22; no. 22; p. 12233
• Main Authors: Tuusa, Jussi, Kokkonen, Nina, Tasanen, Kaisa
• Format: Journal Article
• Language: English
• Published: Switzerland MDPI AG 12.11.2021; MDPI
• Subjects: Alzheimer's disease; Antigens; Autoantigens - immunology; Autoantigens - metabolism; Cancer; Collagen; Collagen Type XVII; Hemidesmosomes - immunology; Hemidesmosomes - metabolism; Humans; Hypotheses; Keratinocytes - immunology; Keratinocytes - metabolism; Lipids; Microscopy; Mutation; Non-Fibrillar Collagens - immunology; Non-Fibrillar Collagens - metabolism; Pemphigoid, Bullous - immunology; Pemphigoid, Bullous - metabolism; Protein Folding; Proteins; Review; Spectrum analysis; bullous pemphigoid; transmembrane collagen; keratinocyte; protein–protein interaction; autoimmune disease; hemidesmosome; intrinsically disordered
• ISSN: 1422-0067; 1661-6596; 1422-0067
• DOI: 10.3390/ijms222212233

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.