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Cleaved-Tumstatin (P1426) rabbit pAb

CAT: 0855-ES1056-01Size: 50 µLDry Ice: NoHazardous: No
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Background
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 r
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 r
Product Name Alternative
COL4A3; Collagen alpha-3 (IV) chain; Goodpasture antigen
UniProt
Q01955
Swiss Prot
Q01955
Reactivity
Human; Monkey
Immunogen
The antiserum was produced against synthesized peptide derived from human Collagen IV alpha3. AA range:1407-1456
Target
Tumstatin
Clonality
Polyclonal
Source
Rabbit
Applications
WB; ELISA
Concentration
1 mg/ml
Dilution
Western Blot: 1/500 - 1/2000. ELISA: 1/40000. Not yet tested in other applications.
Buffer
-20°C/1 year
Molecular Weight
27kD
Storage Conditions
-20°C/1 year
Observed Molecular Weight
27kD
Fragment
IgG
Subcellular Location
Secreted, extracellular space, extracellular matrix, basement membrane. Colocalizes with COL4A4 and COL4A5 in GBM, tubular basement membrane (TBM) and synaptic basal lamina (BL) . .
Other Product Names
COL4A3; Collagen alpha-3 (IV) chain; Goodpasture antigen
Gene ID (Human)
1285

UniProtKB · Q01955

Collagen alpha-3(IV) chain

CO4A3_HUMAN · Homo sapiens

View on UniProt ↗
Primary accession
Q01955
Review status
UniProtKB reviewed (Swiss-Prot)
Gene
COL4A3
Protein existence
1: Evidence at protein level
Organism
Homo sapiens (Human)
Taxonomy ID
9606
Alternative names
—
EC number
—
Processing
Precursor
Secondary accessions
Q53QQ1, Q53R14, Q53RW8, Q9BQT2, Q9NYC4, Q9UDJ9, Q9UDK9, Q9UDL0, Q9UDL1
Protein keywords

Technical term

3D-structureDirect protein sequencingProteomics identificationReference proteome

Disease

Alport syndromeDeafnessDisease variant

Coding sequence diversity

Alternative splicing

Cellular component

Basement membraneExtracellular matrixSecreted

Biological process

Cell adhesion

Domain

CollagenRepeatSignal

PTM

Disulfide bondGlycoproteinHydroxylationPhosphoprotein

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