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Mouse Anti-CBS Recombinant Antibody (30) (CBMAB-C5445-LY)

This product is antibody recognizes CBS. The antibody 30 immunoassay techniques such as: WB, IP, IHC-P, S-ELISA.
See all CBS antibodies

Summary

Host Animal
Mouse
Specificity
Human
Clone
30
Antibody Isotype
IgG2a
Application
WB, IP, IHC-P, S-ELISA

Basic Information

Specificity
Human
Antibody Isotype
IgG2a
Clonality
Monoclonal
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!]

Format
Liquid
Concentration
0.5 mg/ml
Purity
> 95% Purity determined 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
Cystathionine-Beta-Synthase
Introduction
The protein encoded by this gene acts as a homotetramer to catalyze the conversion of homocysteine to cystathionine, the first step in the transsulfuration pathway. The encoded protein is allosterically activated by adenosyl-methionine and uses pyridoxal phosphate as a cofactor. Defects in this gene can cause cystathionine beta-synthase deficiency (CBSD), which can lead to homocystinuria. This gene is a major contributor to cellular hydrogen sulfide production. Multiple alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Feb 2016]
Entrez Gene ID
875
UniProt ID
P35520
Alternative Names
Cystathionine-Beta-Synthase; Serine Sulfhydrase; Beta-Thionase; EC 4.2.1.22; Cystathionine Beta-Synthase; Methylcysteine Synthase; HIP4;
Function
Hydro-lyase catalyzing the first step of the transsulfuration pathway, where the hydroxyl group of L-serine is displaced by L-homocysteine in a beta-replacement reaction to form L-cystathionine, the precursor of L-cysteine. This catabolic route allows the elimination of L-methionine and the toxic metabolite L-homocysteine (PubMed:23981774, PubMed:20506325, PubMed:23974653).
Also involved in the production of hydrogen sulfide, a gasotransmitter with signaling and cytoprotective effects on neurons (By similarity).
Biological Process
Cysteine biosynthetic process Source: BHF-UCL
Cysteine biosynthetic process from serine Source: InterPro
Cysteine biosynthetic process via cystathionine Source: InterPro
DNA protection Source: BHF-UCL
Homocysteine catabolic process Source: UniProtKB
Homocysteine metabolic process Source: UniProtKB
Hydrogen sulfide biosynthetic process Source: UniProtKB
L-cysteine catabolic process Source: UniProtKB
L-serine catabolic process Source: UniProtKB
L-serine metabolic process Source: UniProtKB
Transsulfuration Source: GO_Central
Cellular Location
Cytoplasm; Nucleus
Involvement in disease
Cystathionine beta-synthase deficiency (CBSD): An enzymatic deficiency resulting in altered sulfur metabolism and homocystinuria. The clinical features of untreated homocystinuria due to CBS deficiency include myopia, ectopia lentis, mental retardation, skeletal anomalies resembling Marfan syndrome, and thromboembolic events. Light skin and hair can also be present. Biochemical features include increased urinary homocystine and methionine.

Bublil, E. M., & Majtan, T. (2020). Classical homocystinuria: From cystathionine beta-synthase deficiency to novel enzyme therapies. Biochimie, 173, 48-56.

Rao, G., Murphy, B., Dey, A., Dwivedi, S. K. D., Zhang, Y., Roy, R. V., ... & Mukherjee, P. (2020). Cystathionine beta synthase regulates mitochondrial dynamics and function in endothelial cells. The FASEB Journal, 34(7), 9372-9392.

Sun, S., Weile, J., Verby, M., Wu, Y., Wang, Y., Cote, A. G., ... & Roth, F. P. (2020). A proactive genotype-to-patient-phenotype map for cystathionine beta-synthase. Genome medicine, 12(1), 1-18.

Zheng, F., Han, J., Lu, H., Cui, C., Yang, J., Cui, Q., ... & Geng, B. (2018). Cystathionine beta synthase-hydrogen sulfide system in paraventricular nucleus reduced high fatty diet induced obesity and insulin resistance by brain-adipose axis. Biochimica et Biophysica Acta (BBA)-Molecular Basis of Disease, 1864(10), 3281-3291.

Voskoboeva, E., Semyachkina, A., Yablonskaya, M., & Nikolaeva, E. (2018). Homocystinuria due to cystathionine beta-synthase (CBS) deficiency in Russia: Molecular and clinical characterization. Molecular genetics and metabolism reports, 14, 47-54.

Majtan, T., Krijt, J., Sokolová, J., Křížková, M., Ralat, M. A., Kent, J., ... & Kraus, J. P. (2018). Biogenesis of hydrogen sulfide and thioethers by cystathionine beta-synthase. Antioxidants & redox signaling, 28(4), 311-323.

Yin, W. L., Yin, W. G., Huang, B. S., & Wu, L. X. (2017). Neuroprotective effects of lentivirus-mediated cystathionine-beta-synthase overexpression against 6-OHDA-induced parkinson's disease rats. Neuroscience letters, 657, 45-52.

Majtan, T., Pey, A. L., Gimenez-Mascarell, P., Martínez-Cruz, L. A., Szabo, C., Kožich, V., & Kraus, J. P. (2017). Potential pharmacological chaperones for cystathionine beta-synthase-deficient homocystinuria. Targeting Trafficking in Drug Development, 345-383.

Hendrix, P., Foreman, P. M., Harrigan, M. R., Fisher, W. S., Vyas, N. A., Lipsky, R. H., ... & Griessenauer, C. J. (2017). Association of cystathionine beta-synthase polymorphisms and aneurysmal subarachnoid hemorrhage. Journal of neurosurgery, 128(6), 1771-1777.

Nandi, S. S., & Mishra, P. K. (2017). H 2 S and homocysteine control a novel feedback regulation of cystathionine beta synthase and cystathionine gamma lyase in cardiomyocytes. Scientific reports, 7(1), 1-15.

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

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