Rabbit CBS Polyclonal Antibody | anti-CBS antibody

CBS Antibody

Cat. Num. AAA9402761

• Gene Names: CBS; HIP4
• Reactivity: Human, Mouse, Rat
• Applications: Western Blot, Immunohistochemistry, Immunofluorescence
• Purity: Antibodies were purified by affinity purification using immunogen.
• Synonyms: CBS; Polyclonal Antibody; CBS Antibody; HIP4; anti-CBS antibody

• Host: Rabbit
• Reactivity: Human, Mouse, Rat
• Clonality: Polyclonal
• Specificity: The antibody detects endogenous level of total CBS protein.
• Purity/Purification: Antibodies were purified by affinity purification using immunogen.
• Form/Format: Supplied at 1.0mg/mL in phosphate buffered saline (without Mg2+ and Ca2+), pH 7.4, 150mM NaCl, 0.02% sodium azide and 50% glycerol.
• Concentration: 1.0 mg/ml (varies by lot)
• Sequence Length: 551

• Applicable Applications for anti-CBS antibody: Western Blot (WB), Immunohistochemistry (IHC), Immunofluorescence (IF)
• Application Notes: Western blotting: 1:500 - 1:2000; Immunohistochemistry: 1:50 - 1:100; Immunofluorescence: 1:50 - 1:200
• Immunogen Type: Recombinant Protein
• Immunogen Description: Recombinant protein of human CBS.
• Target Name: CBS
• Preparation and Storage: Store at -20 degree C

Western Blot (WB)

(Western blot analysis of extracts of mouse kidney tissue, using CBS antibody.)

Immunohistochemistry (IHC)

(Immunohistochemical analysis of paraffin-embedded human esophagus using CBS antibody at dilution of 1:100 (400x lens).)

Immunohistochemistry (IHC)

(Immunohistochemical analysis of paraffin-embedded rat kidney using CBS antibody at dilution of 1:100 (400x lens).)

Immunofluorescence (IF)

(Immunofluorescence analysis of U2OS cell using CBS antibody. Blue: DAPI for nuclear staining.)

Related Product Information for anti-CBS antibody

The CBS gene encodes cystathionine beta-synthase, which 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(PMID:11230183). CBS protein is localized in most areas of the brain, but predominantly in the cell bodies and neuronal processes of Purkinje cells and Ammon's horn neurons(PMID:12588964). This antibody is specific to CBS.

Product Categories/Family for anti-CBS antibody

Total protein Ab

NCBI and Uniprot Product Information

• NCBI GI #: 4557415
• NCBI GeneID: 875
• NCBI Accession #: NP_000062.1
• NCBI GenBank Nucleotide #: NM_000071.2
• UniProt Accession #: P35520; Q99425; Q9BWC5; B2R993; D3DSK4
• Molecular Weight: 61,863 Da
• NCBI Official Full Name: cystathionine beta-synthase
• NCBI Official Synonym Full Names: cystathionine-beta-synthase
• NCBI Official Symbol: CBS
• NCBI Official Synonym Symbols: HIP4
• NCBI Protein Information: cystathionine beta-synthase
• UniProt Protein Name: Cystathionine beta-synthase
• Protein Family: Cystathionine beta-synthase
• UniProt Gene Name: CBS
• UniProt Entry Name: CBS_HUMAN

NCBI Description

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]

Uniprot Description

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

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; metal ion binding; ubiquitin protein ligase 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