Anti-CBS Antibody (clone 6A9) IHC-plus; CBS; Beta-thionase; Cystathionine beta-synthase; HIP4; Methylcysteine synthase; Serine sulfhydrase; Cystathionine-beta-synthase; Human CBS

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

Monoclonal

IgG1,k

6A9

Mouse

Human CBS

Protein A Purified

PBS, pH 7.2

Aliquot and store at -20 degree C or -80 degree C. Avoid freeze-thaw cycles.

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.

CBS is involved in transsulfuration pathway. The first step of this pathway, from homocysteine to cystathionine, is catalyzed by CBS. CBS deficiency can cause homocystinuria which affects many organs and tissues including the eyes, the central nervous, skeletal, and vascular systems.

Immunohistochemistry (IHC - Paraffin), Western Blot (WB), ELISA (EIA)

ELISA, IHC-P (5 ug/ml), WB
Usage: Immunohistochemistry: Formalin-fixed paraffin-embedded sections. Sandwich ELISA: Recombinant protein. Western Blot: Recombinant protein.

Anti-CBS antibody IHC of human liver. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 5 ug/ml.

Anti-CBS antibody IHC of human brain, cerebellum. Immunohistochemistry of formalin-fixed, paraffin-embedded tissue after heat-induced antigen retrieval. Antibody concentration 5 ug/ml.

Detection limit for recombinant GST tagged CBS is approximately 0.03 ng/ml as a capture antibody.

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

Chromosomal Location of Human Ortholog: 21q22.3

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

Molecular Function: identical protein binding; protein binding; protein homodimerization activity; enzyme binding; 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; sulfur amino acid metabolic process; L-serine 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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