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Report monoclonal antibody against the collagen type IV (show COL4 Proteins) alpha3NC1 domain as a marker for glomerular disease.
Data show that deletion of tumstatin and TSP1 (show GZMA Proteins) in p53 (show TP53 Proteins)-/- mice leads to increased tumor burden and reduced survival.
the pathogenetic role of USAG-1 (show SOSTDC1 Proteins) in Col4a3-/- mice might involve crosstalk between kidney tubules and the glomerulus and that inhibition of USAG-1 (show SOSTDC1 Proteins) may be a promising therapeutic approach for the treatment of Alport syndrome.
Collagen alpha3(IV) nor alpha4(IV) were detected in the lens capsule 2 weeks postnatal.
Matrix metalloproteinase-9 (show MMP9 Proteins) generated fragments of procollagen, type IV, alpha 3 has endogenous function as integrin-mediated suppressors of pathologic angiogenesis and tumor growth.
Alpha3(IV), alpha4(IV), and alpha5(IV) chains form a complex, which is a heterotrimer, and a defect in complex formation might be one of the molecular mechanisms underlying the pathogenesis of Alport syndrome.
CCR1-mediated recruitment and local activation of macrophages contribute to disease progression in COL4A3-deficient mice. CCR1 is potential therapeutic target for Alport disease or other progressive nephropathies with interstitial macrophage infiltrates.
Upregulation of Lama5 (show LAMA5 Proteins) transcription and concentration of laminin alpha1 and alpha5 within (Alport)collagen alpha3(IV) knockout glomerular basement membrane thickenings contribute to abnormal permeabilities.
A single immunization of highly denatured recombinant mouse collagen IV (show COL4 Proteins) alpha 3 chain noncollagen domain induces severe glomerulonephritis in 100% of Wistar Kyoto rats.
Results showed that COL4A4 (show Col4a4 Proteins) c.1471C>T and COL4A3 c.3418 + 1G>T variants in cis (show CISH Proteins) are pathogenic and co-segregate with the benign familial hematuria. This result suggests that COL4A3 and COL4A4 (show Col4a4 Proteins) digenic mutations in cis (show CISH Proteins) mimicking an autosomal dominant inheritance should be considered as a novel inheritance pattern of benign familial hematuria.
In a large Spanish family with Alport sysndrome, carriers of certain mutations in the COL4A3 gene were more severely affected and had earlier onset of the disease compared to non-carriers of these mutations.
For mutation screening, all exons of COL4A3 and COL4A4 (show Col4a4 Proteins) genes were polymerase chain reaction-amplified and direct sequenced from genomic DNA, and the mutations were analyzed by comparing with members in this family, 100 ethnicitymatched controls and the sequence of COL4A3 and COL4A4 (show Col4a4 Proteins) genes from GenBank. A novel mutation determining a nucleotide change was found, i.e. c.4195 A>T (p.Met1399Leu) at 44th exon of COL4A4 (show Col4a4 Proteins) gene.
Two families showed COL4A3/A4 mutations in cis (show CISH Proteins), mimicking an autosomal dominant inheritance with a more severe phenotype and one showed COL4A3/A4 mutations in trans, mimicking an autosomal recessive inheritance with a less severe phenotype. In a fourth family, a de novo mutation (COL4A5 (show COL4a5 Proteins)) combined with an inherited mutation (COL4A3) triggered a more severe phenotype
Alport syndrome is the result of mutations in any of three type IV collagen (show COL4 Proteins) genes, COL4A3, COL4A4 (show Col4a4 Proteins), or COL4A5 (show COL4a5 Proteins). Because the three collagen chains form heterotrimers, there is an absence of all three proteins in the basement membranes where they are expressed. (Review)
These findings indicate that the heterozygous mutations in COL4A3 or COL4A4 (show Col4a4 Proteins) may cause ESRD on their own, although secondary factors, such as environmental factors or unknown genetic changes, might also contribute to the phenotype of kidney disease in patients with ADAS (show AGPS Proteins).
This study indicates that in our population, the COL4A3 rs55703767 polymorphism decreased the risk of KC. However, the TIMP-1 (show TIMP1 Proteins) rs6609533 polymorphism was associated with an increased risk of KC.
mutations in COL4A3, COL4A4 (show Col4a4 Proteins), and COL4A5 (show COL4a5 Proteins) in Chinese patients with Alport Syndrome
COL4A3 gene expression is negatively regulated by ZEB1 binding to E2 box motifs in the COL4A3 promoter region.
COL4A3 expression is significantly downregulated in human masticatory mucosa during wound healing
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.
type IV collagen alpha 3 chain
, collagen alpha-3(IV) chain
, collagen type IV alpha3 chain
, procollagen, type IV, alpha 3
, collagen, type IV, alpha 3 (Goodpasture antigen)
, collagen IV, alpha-3 polypeptide