CBS, Polyclonal Antibody

Cystathionine beta-synthase; Beta-thionase; Serine sulfhydrase; CBS

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

Polyclonal

Rabbit Ig

Rabbit

This CBS antibody is generated from rabbits immunized with a KLH conjugated synthetic peptide between 104-133 amino acids from the N-terminal region of human CBS.

Peptide Affinity Purified Rabbit Polyclonal Antibody (Pab)

Purified polyclonal antibody supplied in PBS with 0.09% (W/V) sodium azide. This antibody is purified through a protein A column, followed by peptide affinity purification.

Vial Concentration: 0.29 (lot specific)

Maintain refrigerated at 2-8 degree C for up to 6 months. For long term storage store at -20 degree C in small aliquots to prevent 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 acts as a homotetramer to catalyze the conversion of homocysteine to cystathionine, the first step in the transsulfuration pathway. This 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.

Cancer; Metabolism; Signal Transduction

Western Blot (WB), ELISA (EIA), Immunohistochemistry (IHC), Flow Cytometry (FC/FACS), Immunofluorescence (IF)

WB~~1:1000

Western blot analysis of CBS Antibody (N-term) in mouse kidney tissue and Ramos cell line lysates (35ug/lane). CBS (arrow) was detected using the purified Pab.

CBS Antibody (N-term) IHC analysis in formalin fixed and paraffin embedded human hepatocarcinoma followed by peroxidase conjugation of the secondary antibody and DAB staining. This data demonstrates the use of the CBS Antibody (N-term) for immunohistochemistry. Clinical relevance has not been evaluated.

CBS Antibody (N-term) flow cytometric analysis of Ramos cells (bottom histogram) compared to a negative control cell (top histogram).FITC-conjugated goat-anti-rabbit secondary antibodies were used for the analysis.

Western blot analysis of CBS (arrow) using rabbit polyclonal CBS Antibody (N-term). 293 cell lysates (2 ug/lane) either nontransfected (Lane 1) or transiently transfected (Lane 2) with the CBS gene.

Confocal immunofluorescent analysis of CBS Antibody (N-term) with 293 cell followed by Alexa Fluor 488-conjugated goat anti-rabbit lgG (green). Actin filaments have been labeled with Alexa Fluor 555 phalloidin (red).DAPI was used to stain the cell nuclear (blue).

60587

cystathionine-beta-synthase

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

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

Ravel,C., et.al., PLoS ONE 4 (8), E6540 (2009)

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