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Mouse Anti-COL4A4 Recombinant Antibody (CBWJC-2928) (CBMAB-C4071WJ)

This product is a Mouse antibody that recognizes COL4A4. This antibody CBWJC-2928 can be used for immunoassay techniques such as: ELISA, IHC, WB.
See all COL4A4 antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
CBWJC-2928
Antibody Isotype
IgG1, κ
Application
ELISA, IHC, WB

Basic Information

Immunogen
Purified human mu chain protein
Specificity
Human
Antibody Isotype
IgG1, κ
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

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

Concentration
1 mg/mL
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Collagen Type IV Alpha 4 Chain
Introduction
COL4A4 (Collagen Type IV Alpha 4 Chain) is a Protein Coding gene. Diseases associated with COL4A4 include Alport Syndrome, Autosomal Recessive and Hematuria, Benign Familial. Among its related pathways are Primary Focal Segmental Glomerulosclerosis FSGS and Integrin Pathway. Gene Ontology (GO) annotations related to this gene include extracellular matrix structural constituent. An important paralog of this gene is COL4A6.
Entrez Gene ID
1286
UniProt ID
P53420
Alternative Names
Collagen Type IV Alpha 4 Chain; Collagen Of Basement Membrane, Alpha-4 Chain; Collagen, Type IV, Alpha 4; Collagen IV, Alpha-4 Polypeptide; Collagen Alpha-4(IV) Chain; CA44;
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
Collagen fibril organization Source: Reactome
Extracellular matrix organization Source: GO_Central
Glomerular basement membrane development Source: UniProtKB
Cellular Location
Basement membrane. Colocalizes with COL4A4 and COL4A5 in GBM, tubular basement membrane (TBM) and synaptic basal lamina (BL).
Involvement in disease
Alport syndrome 2, autosomal recessive (ATS2):
A syndrome 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.
Hematuria, benign familial (BFH):
An autosomal dominant condition characterized by non-progressive isolated microscopic hematuria that does not result in renal failure. It is characterized pathologically by thinning of the glomerular basement membrane.
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.

Barua, M., & Paterson, A. D. (2021). Population-based studies reveal an additive role of type IV collagen variants in hematuria and albuminuria. Pediatric Nephrology, 1-10.

Imafuku, A., Nozu, K., Sawa, N., Nakanishi, K., & Ubara, Y. (2020). How to resolve confusion in the clinical setting for the diagnosis of heterozygous COL4A3 or COL4A4 gene variants? Discussion and suggestions from nephrologists. Clinical and experimental nephrology, 24(8), 651-656.

Yari, D., Ehsanbakhsh, Z., Validad, M. H., & Langroudi, F. H. (2020). Association of TIMP-1 and COL4A4 gene polymorphisms with keratoconus in an Iranian population. Journal of ophthalmic & vision research, 15(3), 299.

Li, A., Gao, E. Z., Cui, Y. X., Liu, J. H., Lv, X., Wei, X. X., ... & Li, X. J. (2018). Three novel heterozygous COL4A4 mutations result in three different collagen type IV kidney disease phenotypes. Cytogenetic and Genome Research, 154(1), 30-36.

Savige, J. (2018). Should we diagnose autosomal dominant Alport syndrome when there is a pathogenic heterozygous COL4A3 or COL4A4 variant?. Kidney international reports, 3(6), 1239-1241.

Guo, L., Li, D., Dong, S., Wang, D., Yang, B., & Huang, Y. (2017). Mutation analysis of COL4A3 and COL4A4 genes in a Chinese autosomal-dominant Alport syndrome family. Journal of Genetics, 96(2), 389-392.

Liu, J. H., Wei, X. X., Li, A., Cui, Y. X., Xia, X. Y., Qin, W. S., ... & Li, X. J. (2017). Novel mutations in COL4A3, COL4A4, and COL4A5 in Chinese patients with Alport Syndrome. PLoS One, 12(5), e0177685.

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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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