Rabbit anti-Human COL4A3 Polyclonal Antibody | anti-COL4A3 antibody

COL4A3 Antibody (N-Term)

Cat. Num. AAA9216975

• Reactivity: Human
• Applications: Western Blot
• Synonyms: COL4A3; Polyclonal Antibody; COL4A3 Antibody (N-Term); Purified Rabbit Polyclonal Antibody (Pab); Collagen alpha-3(IV) chain; Goodpasture antigen; Tumstatin; anti-COL4A3 antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Sequence Length: 1670

• Applicable Applications for anti-COL4A3 antibody: Western Blot (WB)
• Application Notes: WB~~1:1000
• Antigen Type: Synthesized Peptide
• Preparation and Storage: Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent freeze-thaw cycles.

Western Blot (WB)

(Anti-COL4A3 Antibody (N-Term) at 1:1000 dilution + kidney lysateLysates/proteins at 20 ug per lane. SecondaryGoat Anti-Rabbit IgG, (H+L), Peroxidase conjugated at 1/10000 dilution. Predicted band size : 162 kDaBlocking/Dilution buffer: 5% NFDM/TBST.)

Related Product Information for anti-COL4A3 antibody

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.

Product Categories/Family for anti-COL4A3 antibody

Cancer; Immunology; Signal transduction; Stem cells

References

Mariyama M.,et al.J. Biol. Chem. 269:23013-23017(1994). Leinonen A.,et al.Submitted (OCT-1998) to the EMBL/GenBank/DDBJ databases. Heidet L.,et al.J. Am. Soc. Nephrol. 12:97-106(2001). Hillier L.W.,et al.Nature 434:724-731(2005). Momota R.,et al.FEBS Lett. 424:11-16(1998).

NCBI and Uniprot Product Information

• NCBI GI #: 89142730
• NCBI GeneID: 1285
• NCBI Accession #: NP_000082.2
• NCBI GenBank Nucleotide #: NM_000091.4
• UniProt Accession #: Q01955; Q53QQ1; Q53R14; Q53RW8; Q9BQT2; Q9NYC4; Q9UDJ9; Q9UDK9; Q9UDL0; Q9UDL1
• Molecular Weight: 135,079 Da
• NCBI Official Full Name: collagen alpha-3(IV) chain
• NCBI Official Synonym Full Names: collagen type IV alpha 3
• 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: basement membrane; collagen type IV; endoplasmic reticulum; endoplasmic reticulum lumen; extracellular region; intracellular membrane-bound organelle

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

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

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