Collagen VI Recombinant Rabbit mAb (一抗) - WB,IHC-P,IHC-F,IF | Bioss

bsm-61026R

重组兔单抗、mIHC精品抗体

Collagen VI Recombinant Rabbit mAb

6型胶原重组兔单抗

BTHLM1; BTHLM1A; OPLL; UCHMD1; UCHMD1A; BTHLM1B; PP3610; UCMD1; UCMD1B; BTHLM1C; DYT27; UCMD1C; Col6a-1; Col6a-2; Col6a-3; RGD1565398; CO6A1_HUMAN; COL6A1; CO6A2_HUMAN; COL6A2; CO6A3_HUMAN; COL6A3; CO6A2_MOUSE; CO6A1_MOUSE; collagen type VI alpha 1 chain; collagen, type VI, alpha 1

Rabbit

A synthesized peptide derived from human Collagen VI: 15-250

IgG

Liquid

affinity purified by Protein A

Recombinant

4D10

106 kDa

140 kDa

0.01M TBS(pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.

Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

The collagens are a superfamily of proteins that play a role in maintaining the integrity of various tissues. Collagens are extracellular matrix proteins and have a triple-helical domain as their common structural element. Collagen VI is a major structural component of microfibrils. The basic structural unit of collagen VI is a heterotrimer of the alpha1(VI), alpha2(VI), and alpha3(VI) chains. The alpha2(VI) and alpha3(VI) chains are encoded by the COL6A2 and COL6A3 genes, respectively. The protein encoded by this gene is the alpha 1 subunit of type VI collagen (alpha1(VI) chain). Mutations in the genes that code for the collagen VI subunits result in the autosomal dominant disorder, Bethlem myopathy. [provided by RefSeq, Jul 2008]

COL6A1

Collagen alpha-1(VI) chain

Trimers composed of three different chains: alpha-1(VI), alpha-2(VI), and alpha-3(VI) or alpha-5(VI) or alpha-6(VI).

Secreted, extracellular space, extracellular matrix (By similarity).

Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.

Bethlem myopathy (BM) [MIM:158810]: A benign autosomal dominant proximal myopathy characterized by early childhood onset and joint contractures most frequently affecting the elbows and ankles. Note=The disease is caused by mutations affecting the gene represented in this entry.
Ullrich congenital muscular dystrophy (UCMD) [MIM:254090]: UCMD is 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. Note=The disease is caused by mutations affecting the gene represented in this entry.

Belongs to the type VI collagen family.
Contains 3 VWFA domains.

Collagen VI acts as a cell-binding protein.