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anti-COL4A3 antibody :: Rabbit anti-Human, Mouse COL4A3 Polyclonal Antibody

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Catalog # MBS2525973
Unit / Price
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  0.02 mL  /  $110 +1 FREE 8GB USB
  0.06 mL  /  $150 +1 FREE 8GB USB
  0.12 mL  /  $225 +1 FREE 8GB USB
  0.2 mL  /  $360 +1 FREE 8GB USB
Product Name

COL4A3, Polyclonal Antibody

Popular Item
Full Product Name

COL4A3 Polyclonal Antibody

Product Synonym Names
COL4A3,Collagen alpha-3(IV) chain,Goodpasture antigen
Research Use Only
For Research Use Only. Not for use in diagnostic procedures.
MBS2525973 Technical Datasheet
OMIM
104200
3D Structure
ModBase 3D Structure for Q01955
Clonality
Polyclonal
Isotype
IgG
Host
Rabbit
Species Reactivity
Human, Mouse
Purity/Purification
Affinity purification
Immunogen
Synthetic peptide of human COL4A3
Buffer
PBS with 0.05% sodium azide, 50% glycerol, pH7.3
Preparation and Storage
Store at -20°C. Avoid freeze / thaw cycles.
ISO Certification
Manufactured in an ISO 9001:2015 Certified Laboratory.
Other Notes
Small volumes of anti-COL4A3 antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.
Related Product Information for
anti-COL4A3 antibody
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.
Product Categories/Family for anti-COL4A3 antibody
Applications Tested/Suitable for anti-COL4A3 antibody
ELISA (EIA), Immunohistochemistry (IHC)
Application Notes for anti-COL4A3 antibody
IHC: 1:25-1:100

Immunohistochemistry (IHC) of anti-COL4A3 antibody
Immunohistochemistry of paraffin-embedded Human ovarian cancer tissue using COL4A3 Polyclonal Antibody at dilution 1:20
anti-COL4A3 antibody Immunohistochemistry (IHC) (IHC) image
Immunohistochemistry (IHC) of anti-COL4A3 antibody
Immunohistochemistry of paraffin-embedded Human thyroid cancer tissue using COL4A3 Polyclonal Antibody at dilution 1:20
anti-COL4A3 antibody Immunohistochemistry (IHC) (IHC) image
NCBI/Uniprot data below describe general gene information for COL4A3. It may not necessarily be applicable to this product.
NCBI GI #
NCBI GeneID
NCBI Accession #
NCBI GenBank Nucleotide #
UniProt Primary Accession #
UniProt Secondary Accession #
UniProt Related Accession #
Molecular Weight
135,079 Da
NCBI Official Full Name
collagen alpha-3(IV) chain
NCBI Official Synonym Full Names
collagen, type IV, alpha 3 (Goodpasture antigen)
NCBI Official Symbol
COL4A3  [Similar Products]
NCBI Protein Information
collagen alpha-3(IV) chain; collagen alpha-3(IV) chain; collagen IV, alpha-3 polypeptide; tumstatin
UniProt Protein Name
Collagen alpha-3(IV) chain
UniProt Synonym Protein Names
Goodpasture antigenTumstatin
Protein Family
UniProt Gene Name
COL4A3  [Similar Products]
UniProt Entry Name
CO4A3_HUMAN
NCBI Summary for COL4A3
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 Comments for COL4A3
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; negative regulation of cell proliferation; collagen catabolic process; extracellular matrix disassembly; 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
Precautions
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Disclaimer
While every efforts were made to ensure the accuracy of the information provided in this datasheet, MyBioSource will not be liable for any omissions or errors contained herein. MyBioSource reserves the right to make changes to this datasheet at any time without prior notice.

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