Rabbit anti-Human COL6A1 Polyclonal Antibody | anti-COL6A1 antibody

COL6A1, CT (Collagen alpha-1(VI) Chain) (PE)

Cat. Num. AAA6469885

• Gene Names: COL6A1; OPLL
• Reactivity: Human
• Applications: Western Blot, ELISA
• Purity: Purified by Ammonium Sulfate Precipitation.
• Synonyms: COL6A1; Polyclonal Antibody; CT (Collagen alpha-1(VI) Chain) (PE); anti-COL6A1 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human COL6A1.
• Purity/Purification: Purified by Ammonium Sulfate Precipitation.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with R-Phycoerythrin (PE).

• Applicable Applications for anti-COL6A1 antibody: Western Blot (WB), ELISA (EIA)
• Application Notes: WB: 1:50-1:100; ELISA: 1:1,000; Applications are based on unconjugated antibody.
• Immunogen: Synthetic peptide selected from the C-terminal region of human COL6A1 (KLH).
• Conjugate: PE
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: PE conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(Western blot analysis of COL6A1 antibody (C-term) in Y79 cell line lysates (35ug/lane). COL6A1 (arrow) was detected using the purified Pab.)

Immunohistochemistry (IHC)

(Formalin-fixed and paraffin-embedded human hepatocarcinoma with COL6A1 Antibody (C-term), which was peroxidase-conjugated to the secondary antibody, followed by DAB staining. This data demonstrates the use of this antibody for immunohistochemistry; clinical relevance has not been evaluated.)

Immunofluorescence (IF)

(Confocal immunofluorescent analysis of COL6A1 Antibody (C-term) with A2058 cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green).DAPI was used to stain the cell nuclear (blue).)

Flow Cytometry (FC/FACS)

(Flow cytometric analysis of 293 cells using COL6A1 Antibody (C-term)(bottom histogram) compared to a negative control cell (top histogram). FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.)

Product Categories/Family for anti-COL6A1 antibody

Antibodies; Collagen

References

1. Martoni, E., Hum. Mutat. 30(5), E662-E672 (2009). 2. Kim, T.H., J. Rheumatol. 35(9), 1849-1852 (2008). 3. Fujita, A., Genet. Mol. Res. 7(2), 371-378 (2008).

NCBI and Uniprot Product Information

• NCBI GI #: 87196339
• NCBI GeneID: 1291
• NCBI Accession #: NP_001839
• NCBI GenBank Nucleotide #: NM_001848
• UniProt Accession #: P12109; O00117; O00118; Q14040; Q14041; Q16258; Q7Z645; Q9BSA8
• Molecular Weight: 108,529 Da
• NCBI Official Full Name: collagen alpha-1(VI) chain
• NCBI Official Synonym Full Names: collagen, type VI, alpha 1
• NCBI Official Symbol: COL6A1
• NCBI Official Synonym Symbols: OPLL
• NCBI Protein Information: collagen alpha-1(VI) chain; collagen alpha-1(VI) chain; alpha 1 (VI) chain (61 AA); collagen VI, alpha-1 polypeptide
• UniProt Protein Name: Collagen alpha-1(VI) chain
• Protein Family: Collagen
• UniProt Gene Name: COL6A1
• UniProt Entry Name: CO6A1_HUMAN

Uniprot Description

COL6A1: Collagen VI acts as a cell-binding protein. Defects in COL6A1 are a cause of Bethlem myopathy (BM). BM is a rare autosomal dominant proximal myopathy characterized by early childhood onset (complete penetrance by the age of 5) and joint contractures most frequently affecting the elbows and ankles. Defects in COL6A1 are a cause of Ullrich congenital muscular dystrophy (UCMD); also known as Ullrich scleroatonic muscular dystrophy. UCMD is an autosomal recessive 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. Belongs to the type VI collagen family.

Protein type: Extracellular matrix; Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 21q22.3

Cellular Component: extracellular matrix; proteinaceous extracellular matrix; protein complex; membrane; lysosomal membrane; endoplasmic reticulum lumen; extracellular region; sarcolemma; collagen type VI

Molecular Function: platelet-derived growth factor binding

Biological Process: osteoblast differentiation; axon guidance; extracellular matrix disassembly; collagen catabolic process; extracellular matrix organization and biogenesis; cell adhesion

Disease: Bethlem Myopathy; Ullrich Congenital Muscular Dystrophy