CBS, Polyclonal Antibody

Cystathionine beta-synthase; cystathionine-beta-synthase; AI047524 antibody; AI303044 antibody; Beta thionase antibody; Beta-thionase antibody; Cbs antibody; Cbs cystathionine beta-synthase antibody; CBS_HUMAN antibody; Cystathionine beta synthase antibody; Cystathionine beta-synthase antibody; EC 4.2.1.22 antibody; HIP 4 antibody; HIP4 antibody; Methylcysteine synthase antibody; MGC18856 antibody; MGC18895 antibody; MGC37300 antibody; OTTHUMP00000109416 antibody; OTTHUMP00000109418 antibody; Serine sulfhydrase antibody

For Research Use Only. Not for use in diagnostic procedures.

Polyclonal

IgG

Rabbit

Immunogen affinity purified.

Lyophilized

At -20 degree C for one year. After reconstitution, at 4 degree C for one month. It can also be aliquotted and stored frozen at -20 degree C for a longer time. Avoid repeated freezing and thawing.

Manufactured in an ISO 13485:2003 and EN ISO 13485:2012 Certified Laboratory.

Small volumes of anti-CBS antibody vial(s) may occasionally become entrapped in the seal of the product vial during shipment and storage. If necessary, briefly centrifuge the vial on a tabletop centrifuge to dislodge any liquid in the container`s cap. Certain products may require to ship with dry ice and additional dry ice fee may apply.

Description: Rabbit IgG polyclonal antibody for Cystathionine beta-synthase(CBS) detection. Tested with WB, IHC-P in Human.
Background: CBS, Cystathionine Beta-Synthase, catalyzes the first irreversible step of transsulfuration. The CBS enzyme is a homotetramer of 63-kD subunits and requires pyridoxal phosphate and heme for activity. The CBS gene, which is mapped to chromosome 21q22, contains 23 exons, ranging in size from 42 to 299 bp. The human CBS protein can substitute for the endogenous yeast CBS protein in Saccharomyces cerevisiae. The catalytic domain of the CBS protein is located in the N-terminal 409 amino acids, and a regulatory domain is located in the C-terminal 142 amino acids.A mutation that deletes the C-terminal 145 amino acids of CBS could restore activity of several CBS mutant alleles found in homocystinurics.

Western Blot (WB), Immunohistochemistry (IHC) Paraffin

Anti-CBS antibody, MBS175681, Western blotting
Lane 1: SMMC Cell Lysate
Lane 2: U87 Cell Lysate
Lane 3: HELA Cell Lysate
Lane 4: MM231 Cell Lysate

Anti-CBS antibody, MBS175681, IHC(P)
IHC(P): Human Liver Cancer Tissue

61,863 Da

cystathionine-beta-synthase

cystathionine beta-synthase; beta-thionase; serine sulfhydrase; methylcysteine synthase

Cystathionine beta-synthase

CBS_HUMAN

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. Multiple alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, May 2010]

CBS: Only known pyridoxal phosphate-dependent enzyme that contains heme. Important regulator of hydrogen sulfide, especially in the brain, utilizing cysteine instead of serine to catalyze the formation of hydrogen sulfide. Hydrogen sulfide is a gastratransmitter with signaling and cytoprotective effects such as acting as a neuromodulator in the brain to protect neurons against hypoxic injury. Defects in CBS are the cause of cystathionine beta- synthase deficiency (CBSD). CBSD is 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. Belongs to the cysteine synthase/cystathionine beta- synthase family. 2 isoforms of the human protein are produced by alternative splicing.

Protein type: Lyase; Other Amino Acids Metabolism - selenoamino acid; EC 4.2.1.22; Amino Acid Metabolism - glycine, serine and threonine; Amino Acid Metabolism - cysteine and methionine

Chromosomal Location of Human Ortholog: 21q22.3

Cellular Component: intracellular membrane-bound organelle; nucleolus; nucleus; cytosol

Molecular Function: identical protein binding; protein binding; enzyme binding; protein homodimerization activity; cystathionine beta-synthase activity; ubiquitin protein ligase binding; metal ion binding; heme binding; pyridoxal phosphate binding

Biological Process: cysteine biosynthetic process via cystathionine; homocysteine catabolic process; L-serine metabolic process; sulfur amino acid metabolic process; transsulfuration; cysteine biosynthetic process from serine; homocysteine metabolic process; L-serine catabolic process; L-cysteine catabolic process

Disease: Homocystinuria Due To Cystathionine Beta-synthase Deficiency

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