Rabbit Collagen IV alpha 3 Polyclonal Antibody | anti-COL4A3 antibody

Collagen IV alpha 3 antibody

Cat. Num. AAA5300091

• Applications: Immunohistochemistry, Immunofluorescence, ELISA
• Synonyms: Collagen IV alpha 3; Polyclonal Antibody; Collagen IV alpha 3 antibody; Polyclonal Collagen IV alpha 3; Anti-Collagen IV alpha 3; COL4A-3; Collagen alpha-3(IV) chain; anti-COL4A3 antibody

• Host: Rabbit
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Collagen IV alpha 3 antibody detects endogenous levels of total Collagen IV alpha3 protein
• Form/Format: Purified IgG supplied in PBS (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol
• Concentration: 1 mg/ml (varies by lot)
• Sequence Length: 1480

• Applicable Applications for anti-COL4A3 antibody: Immunohistochemistry (IHC), Immunofluorescence (IF), ELISA (EIA)
• Application Notes: IHC: 1:50-1:100; IF: 1:100-1:500; ELISA: 1:5000
• Biological Significance: Collagen IV alpha 3 makes the alpha3(IV) chain of type IV collagen. This chain combines with two other types of alpha (IV) chains (the alpha4 and alpha5 chains) to make a complete type IV collagen molecule.
• Immunogen: Collagen IV alpha 3 antibody was raised in Rabbit using synthesized peptide derived from internal of human Collagen IV alpha3 as the immunogen
• Preparation and Storage: Store in small aliquots at -20 degree C. Avoid repeated freeze/thaw cycles

Immunohistochemistry (IHC)

(Immunohistochemistry analysis of paraffin-embedded human brain tissue using Collagen IV alpha 3 antibody)

Related Product Information for anti-COL4A3 antibody

Rabbit polyclonal Collagen IV alpha 3 antibody

Product Categories/Family for anti-COL4A3 antibody

Cell Biology; Purified Polyclonal Antibodies

NCBI and Uniprot Product Information

• NCBI GI #: 119591261
• NCBI GeneID: 1285
• Molecular Weight: 135,079 Da
• NCBI Official Full Name: collagen, type IV, alpha 3 (Goodpasture antigen), isoform CRA_h
• NCBI Official Synonym Full Names: collagen, type IV, alpha 3 (Goodpasture antigen)
• NCBI Official Symbol: COL4A3
• NCBI Protein Information: collagen alpha-3(IV) chain
• UniProt Protein Name: Collagen alpha-3(IV) chain
• Protein Family: Collagen
• UniProt Gene Name: COL4A3
• UniProt Entry Name: CO4A3_HUMAN

NCBI Description

Type IV collagen, the major structural component of basement membranes, is a multimeric protein composed of 3 alpha subunits. These subunits are encoded by 6 different genes, alpha 1 through alpha 6, each of which can form a triple helix structure with 2 other subunits to form type IV collagen. This gene encodes alpha 3. In the Goodpasture syndrome, autoantibodies bind to the collagen molecules in the basement membranes of alveoli and glomeruli. The epitopes that elicit these autoantibodies are localized largely to the non-collagenous C-terminal domain of the protein. A specific kinase phosphorylates amino acids in this same C-terminal region and the expression of this kinase is upregulated during pathogenesis. This gene is also linked to an autosomal recessive form of Alport syndrome. The mutations contributing to this syndrome are also located within the exons that encode this C-terminal region. Like the other members of the type IV collagen gene family, this gene is organized in a head-to-head conformation with another type IV collagen gene so that each gene pair shares a common promoter. [provided by RefSeq, Jun 2010]

Uniprot Description

COL4A3: Type IV collagen is the major structural component of glomerular basement membranes (GBM), forming a 'chicken-wire' meshwork together with laminins, proteoglycans and entactin/nidogen. Autoantibodies against the NC1 domain of alpha 3(IV) are found in Goodpasture syndrome, an autoimmune disease of lung and kidney. Defects in COL4A3 are a cause of Alport syndrome autosomal recessive (APSAR). APSAR is characterized by progressive glomerulonephritis, glomerular basement membrane defects, renal failure, sensorineural deafness and specific eye abnormalities (lenticonous and macular flecks). The disorder shows considerable heterogeneity in that families differ in the age of end-stage renal disease and the occurrence of deafness. Defects in COL4A3 are a cause of benign familial hematuria (BFH); also known as thin basement membrane nephropathy. BFH is characterized by persistent hematuria, an electron microscopically detectable thin glomerular basement membrane (GBM) and an autosomal dominant mode of inheritance. Renal function remains normal. In children, differentiation between BFH and AS can be difficult, because both disorders are manifested by persistent hematuria and thin GBM at that age. Defects in COL4A3 are a cause of Alport syndrome autosomal dominant (APSAD). Alport syndrome is characterized by progressive glomerulonephritis, glomerular basement membrane defects, renal failure, sensorineural deafness and specific eye abnormalities (lenticonous and macular flecks). The disorder shows considerable heterogeneity in that families differ in the age of end-stage renal disease and the occurrence of deafness. Belongs to the type IV collagen family. 5 isoforms of the human protein are produced by alternative splicing.

Protein type: Secreted, signal peptide; Secreted

Chromosomal Location of Human Ortholog: 2q36-q37

Cellular Component: endoplasmic reticulum lumen; collagen type IV; extracellular region; basement membrane

Molecular Function: metalloendopeptidase inhibitor activity; integrin binding; protein binding; extracellular matrix structural constituent; structural molecule activity

Biological Process: caspase activation; axon guidance; extracellular matrix organization and biogenesis; blood circulation; glomerular basement membrane development; extracellular matrix disassembly; negative regulation of cell proliferation; collagen catabolic process; cell proliferation; negative regulation of angiogenesis; sensory perception of sound; cell surface receptor linked signal transduction; cell adhesion

Disease: Hematuria, Benign Familial; Alport Syndrome, Autosomal Dominant; Alport Syndrome, Autosomal Recessive