Mouse CBS Monoclonal Antibody | anti-CBS antibody

CBS (Cystathionine-beta-Synthase, HIP4) (FITC)

Cat. Num. AAA6177464

• Gene Names: CBS; HIP4
• Applications: Immunohistochemistry, Western Blot
• Purity: Purified
• Synonyms: CBS; Monoclonal Antibody; CBS (Cystathionine-beta-Synthase; HIP4) (FITC); Cystathionine-beta-Synthase; HIP4; anti-CBS antibody

• Host: Mouse
• Clonality: Monoclonal
• Isotype: IgG2a,k
• Clone Number: 6B8
• Specificity: Recognizes CBS.
• Purity/Purification: Purified
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Fluorescein isothiocyanate (FITC).

• Applicable Applications for anti-CBS antibody: Immunohistochemistry (IHC), Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: CBS (NP_000062, 1aa-100aa) partial recombinant protein with GST tag. MW of the GST tag alone is 26kD.
• Immunogen Sequence: MPSETPQAEVGPTGCPHRSGPHSAKGSLEKGSPEDKEAKEPLWIRPDAPSRCTWQLGRPASESPHHHTAPAKSPKILPDILKKIGDTPMVRINKIGKKFG
• Conjugate: FITC
• Preparation and Storage: Store product at 4 degree C if to be used immediately within two weeks. For long-term storage, aliquot to avoid repeated freezing and thawing and store at -20 degree C. Aliquots are stable at -20 degree C for 12 months after receipt. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer.; ; FITC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap.

Testing Data

(Detection limit for recombinant GST tagged CBS is approximately 0.3ng/ml as a capture antibody.)

Immunohistochemistry (IHC)

(Immunoperoxidase of monoclonal antibody to CBS on formalin-fixed paraffin-embedded human liver. [antibody concentration 3 ug/ml])

Immunohistochemistry (IHC)

(Immunoperoxidase of monoclonal antibody to CBS on formalin-fixed paraffin-embedded human liver. [antibody concentration 3 ug/ml])

Related Product Information for anti-CBS antibody

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. [provided by RefSeq]

Product Categories/Family for anti-CBS antibody

Antibodies; Enzymes; Synthase

NCBI and Uniprot Product Information

• NCBI GI #: 4557415
• NCBI GeneID: 875
• NCBI Accession #: NP_000062
• NCBI GenBank Nucleotide #: NM_000071
• 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; beta-thionase; methylcysteine synthase; serine sulfhydrase
• UniProt Protein Name: Cystathionine beta-synthase
• Protein Family: Cystathionine beta-synthase
• UniProt Gene Name: CBS
• UniProt Entry Name: CBS_HUMAN

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

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