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Mouse Anti-COL4A6 Recombinant Antibody (CBWJC-2936) (V2LY-1206-LY680)


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Summary

Host Animal
Mouse
Specificity
Mouse
Clone
CBWJC-2936
Antibody Isotype
IgG2a, κ
Application
ELISA

Basic Information

Immunogen
Full-length recombinant COL4A6.
Host Species
Mouse
Specificity
Mouse
Antibody Isotype
IgG2a, κ
Clonality
Monoclonal Antibody

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Buffer
PBS
Preservative
None
Concentration
Batch dependent
Purity
>95% as determined by analysis by SDS-PAGE
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
Collagen Type IV Alpha 6 Chain
Entrez Gene ID
94216
UniProt ID
B1AVK5
Function
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.
Biological Process
Cell adhesion Source: UniProtKB-KW
Collagen-activated tyrosine kinase receptor signaling pathway Source: GO_Central
Collagen fibril organization Source: Reactome
Extracellular matrix organization Source: GO_Central
Cellular Location
Basement membrane
Involvement in disease
Deletions covering the N-terminal regions of COL4A5 and COL4A6, which are localized in a head-to-head manner, are found in the chromosome Xq22.3 centromeric deletion syndrome. This results in a phenotype with features of diffuse leiomyomatosis and Alport syndrome (DL-ATS).
Deafness, X-linked, 6 (DFNX6):
A non-syndromic form of sensorineural hearing loss with prelingual onset. Sensorineural deafness results from damage to the neural receptors of the inner ear, the nerve pathways to the brain, or the area of the brain that receives sound information.
PTM
Prolines at the third position of the tripeptide repeating unit (G-X-Y) are hydroxylated in some or all of the chains.
Type IV collagens contain numerous cysteine residues which are involved in inter- and intramolecular disulfide bonding. 12 of these, located in the NC1 domain, are conserved in all known type IV collagens.
The trimeric structure of the NC1 domains is stabilized by covalent bonds between Lys and Met residues.
More Infomation

O’Brien, A., Aw, W. Y., Tee, H. Y., Naegeli, K. M., Bademci, G., Tekin, M., ... & Pandya, A. (2022). Confirmation of COL4A6 variants in X-linked nonsyndromic hearing loss and its clinical implications. European Journal of Human Genetics, 30(1), 7-12.

Tang, S., Yonezawa, T., Maeda, Y., Ono, M., Maeba, T., Miyoshi, T., ... & Oohashi, T. (2021). Lack of collagen α6 (IV) chain in mice does not cause severe-to-profound hearing loss or cochlear malformation, a distinct phenotype from nonsyndromic hearing loss with COL4A6 missense mutation. Plos one, 16(4), e0249909.

Thomas, M. H., Gui, Y., Garcia, P., Karout, M., Gomez Ramos, B., Jaeger, C., ... & Buttini, M. (2021). Quantitative trait locus mapping identifies a locus linked to striatal dopamine and points to collagen IV alpha‐6 chain as a novel regulator of striatal axonal branching in mice. Genes, Brain and Behavior, 20(8), e12769.

Mamoor, S. (2021). Differential expression of collagen type IV alpha 6 chain in human epithelial ovarian cancer.

Ma, J. B., Bai, J. Y., Zhang, H. B., Gu, L., He, D., & Guo, P. (2020). Downregulation of collagen COL4A6 is associated with prostate cancer progression and metastasis. Genetic Testing and Molecular Biomarkers, 24(7), 399-408.

Komori, T., Ono, M., Hara, E. S., Ueda, J., Nguyen, H. T. T., Nguyen, H. T., ... & Oohashi, T. (2018). Type IV collagen α6 chain is a regulator of keratin 10 in keratinization of oral mucosal epithelium. Scientific reports, 8(1), 1-11.

Wang, X., Li, W., Wei, K., Xiao, R., Wang, J., Ma, H., ... & Li, C. (2018). Missense mutations in COL4A5 or COL4A6 genes may cause cerebrovascular fibromuscular dysplasia: case report and literature review. Medicine, 97(30).

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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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