2017
DOI: 10.1186/s13104-017-2674-x
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Authentication of collagen VI antibodies

Abstract: BackgroundCollagen VI is a ubiquitously-expressed macromolecule that forms unique microfibrillar assemblies in the extracellular matrix. Mutations in the COL6A1, COL6A2 and COL6A3 genes result in congenital muscular dystrophy, arguing that collagen is critical for skeletal muscle development and function. Antibodies against collagen VI are important clinical and diagnostic tools in muscular dystrophy. They are used to confirm genetic findings by detecting abnormalities in the distribution, organization and ove… Show more

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Cited by 7 publications
(3 citation statements)
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“…In skeletal muscle, type VI collagen is present in the ECM where it functions to anchor the basement membrane to underlying interstitial tissues [99]. Mutations in the COL6A1, COL6A2, and COL6A3 genes lead to a continuous spectrum of disorders characterized by muscle weakness and connective tissue abnormalities [100].…”
Section: Type VI Collagenmentioning
confidence: 99%
“…In skeletal muscle, type VI collagen is present in the ECM where it functions to anchor the basement membrane to underlying interstitial tissues [99]. Mutations in the COL6A1, COL6A2, and COL6A3 genes lead to a continuous spectrum of disorders characterized by muscle weakness and connective tissue abnormalities [100].…”
Section: Type VI Collagenmentioning
confidence: 99%
“…To unambiguously identify COLVI as the AL59-decorating polymer, we performed IEM applying a COLVI-specific 61 polyclonal antibody followed by gold-conjugated secondary antibody staining of the same extract used for cryo-EM and cryo-ET sample preparations. The IEM images revealed single amyloid fibrils, amyloid clusters and free COLVI polymers tagged by gold (Fig.…”
Section: Colvi Forms Helical Superstructures With Al59 Fibrilsmentioning
confidence: 99%
“…Kollagen VI als perlschnurartiges Kollagen ist Bestandteil der extrazellulären Matrix der Skelettmuskeln und verankert die Basalmembran mit dem interstitiellen Gewebe (Endicott et al 2017) . Die autosomal dominanten und rezessiv vererbten Genmutationen sind mit der kongenitalen Ullrich-Muskeldystrophie, der Bethlem-Myopathie oder einer starken Keloid-Bildung nach Hautverletzungen verbunden (Echeverria et al 2017).…”
Section: Differentialexpression Der Gene Vonunclassified