Mouse Anti-CBS Recombinant Antibody (6A9) (CBMAB-C8065-LY)

Name: Mouse Anti-CBS Recombinant Antibody (6A9)
SKU: CBMAB-C8065-LY
Rating: 5 (1 reviews)

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Published Data

Mouse Anti-CBS Recombinant Antibody (6A9) (CBMAB-C8065-LY) in WB

Severely elevated Hcy does not induce CBS or SAHH expression in female Cgl null mice. Western blotting analysis of hepatic SAHH, CBS and CGL protein expression levels in male and female WT and Cgl null mice. Blotting and immunostaining were performed as described in the Materials and Methods section, In this experiment and all subsequent blots, the relative intensities of protein bands were quantified using Quantity One version 4.6.5 software (Bio Rad). Signal intensity from target bands were calculated relative to GAPDH signal intensity. All blots shown are representative of a minimum of two independent experiments. Values shown represent the mean±s.d., *P<0.05. ...View More

Jiang, H., Hurt, K. J., Breen, K., Stabler, S. P., Allen, R. H., Orlicky, D. J., & Maclean, K. N. (2015). Sex-specific dysregulation of cysteine oxidation and the methionine and folate cycles in female cystathionine gamma-lyase null mice: a serendipitous model of the methylfolate trap. Biology open, 4(9), 1154-1162.

Datasheet

SDS

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Datasheet Target References Q & As Review & reward Protocols Associated Products

Basic Information

Host Animal

Mouse

Clone

6A9

Application

ELISA, IHC-P, WB, IF

Immunogen

CBS (1 a.a. ~ 100 a.a) partial recombinant protein with GST tag.

Host Species

Mouse

Specificity

Human

Antibody Isotype

IgG1, κ

Clonality

Monoclonal

Application Notes

The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Application	Note
IHC-P	3 μg/ml
IF(ICC)	10 μg/ml

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

Format

Liquid

Buffer

PBS

Preservative

None

Concentration

Batch dependent

Storage

Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

More Infomation

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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Please try the standard protocols which include: protocols, troubleshooting and guide.

Enzyme-linked Immunosorbent Assay (ELISA)
Flow Cytometry
Immunofluorescence (IF)
Immunohistochemistry (IHC)
Immunoprecipitation (IP)
Western Blot (WB)
Enzyme Linked Immunospot (ELISpot)
Proteogenomic
Other Protocols

Antibody Pairs

CBS Matched Antibody Pair (192) (CAT#: APMAB-192LY)

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Isotype control

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

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