Human CBS ELISA Kit (V2LY-0626-LY3826)

Name: Human CBS ELISA Kit
SKU: V2LY-0626-LY3826
Rating: 5 (1 reviews)

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Tested Data

Datasheet

SDS

Request for COA

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

Basic Information

Sensitivity

0.027 ng/mL

Detection Range

0.05-20 ng/mL

Sample Type

Serum, Plasma, cell culture supernates

Specificity

Human

Assay Type

Sandwich

Reactivity

Human

Assay Time

1.5 h

Molecule Mass

60.6 kDa

Components

Pre-coated ELISA Plate: 12 wells * 8 detachable strips
Standard solution: 0.5ml x1
Standard diluent: 3ml x1
Streptavidin-HRP: 6ml x1
Stop solution: 6ml x1
Substrate solution A: 6ml x1
Substrate solution B: 6ml x1
Wash buffer concentrate (25x): 20ml x1
Biotinylated antibody: 1ml x1

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

Storage

Store at 2-8°C

More Infomation

Target

Full Name

Cystathionine-Beta-Synthase

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

Alternative Versions

Rat Cbs ELISA Kit (CAT#: V2LY-0626-LY2168)

Mouse Cbs ELISA Kit (CAT#: V2LY-0626-LY3378)

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

Antibody Pairs

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

Related Products

Mouse Anti-CBS Recombinant Antibody (CBXC-1557) (CAT#: CBMAB-C4944-CQ)

Mouse Anti-CBS Recombinant Antibody (CBYY-C1032) (CAT#: CBMAB-C2469-YY)

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

Rabbit Anti-CBS Recombinant Antibody (D8F2P) (CAT#: CBMAB-CP0153-LY)

Mouse Anti-CBS Recombinant Antibody (3D10) (CAT#: CBMAB-C5850-LY)

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

Mouse Anti-CBS Recombinant Antibody (CBXC-0095) (CAT#: CBMAB-C4942-CQ)

Rabbit Anti-CBS Recombinant Antibody (30) (CAT#: CBMAB-C5445-LY)

Mouse Anti-CBS Recombinant Antibody (CBXC-2291) (CAT#: CBMAB-C4943-CQ)

Mouse Cbs ELISA Kit (CAT#: V2LY-0626-LY3378)

Isotype control

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

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