Mouse Anti-COL12A1 Recombinant Antibody (CBYY-C3117) (CBMAB-C4560-YY) in Immunoblot
Immunoblot analyses of control transfected GFP-lamin A (LA) and GFP-progerin fibroblasts as well as GFP-progerin fibroblasts transfected with myc-LAP2α in total and soluble ECM (DOC) fractions using anti-Col12A1, anti-Col1A1, and anti-Timp2 antibodies, with Col4A1 as a loading control for soluble fraction and γ-tubulin as a control for total cell lysate.
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Vidak, S., Kubben, N., Dechat, T., & Foisner, R. (2015). Proliferation of progeria cells is enhanced by lamina-associated polypeptide 2α (LAP2α) through expression of extracellular matrix proteins. Genes & development, 29(19), 2022-2036.
Distributed under Open Access license CC BY 4.0, without modification.
Type XII collagen interacts with type I collagen-containing fibrils, the COL1 domain could be associated with the surface of the fibrils, and the COL2 and NC3 domains may be localized in the perifibrillar matrix.
Ullrich congenital muscular dystrophy 2 (UCMD2): A form of Ullrich muscular dystrophy, a congenital myopathy characterized by muscle weakness and multiple joint contractures, generally noted at birth or early infancy. The clinical course is more severe than in Bethlem myopathy. Bethlem myopathy 2 (BTHLM2): A form of Bethlem myopathy, a benign proximal myopathy characterized by early childhood onset and joint contractures most frequently affecting the elbows and ankles. BTHLM2 inheritance is autosomal dominant.
PTM
The triple-helical tail is stabilized by disulfide bonds at each end. Hydroxylation on proline residues within the sequence motif, GXPG, is most likely to be 4-hydroxy as this fits the requirement for 4-hydroxylation in vertebrates. Isoform 1 O-glycosylation; glycosaminoglycan of chondroitin-sulfate type.
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