Metabolism Antibodies 3
Anti-CBS Antibody (CAB11612)
- SKU:
- CAB11612
- Product Type:
- Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Antibody Type:
- Monoclonal Antibody
- Research Area:
- Metabolism
Description
| Antibody Name: | Anti-CBS Antibody |
| Antibody SKU: | CAB11612 |
| Antibody Size: | 20uL, 50uL, 100uL |
| Application: | WB IHC IF |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | A synthesized peptide derived from human CBS |
| Application: | WB IHC IF |
| Recommended Dilution: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 IF 1:50 - 1:200 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | HepG2, MCF7, Mouse liver, Mouse kidney, Rat liver |
| Immunogen: | A synthesized peptide derived from human CBS |
| Purification Method: | Affinity purification |
| Storage Buffer: | Store at -20°C. Avoid freeze / thaw cycles. Buffer: PBS with 0.02% sodium azide, 0.05% BSA, 50% glycerol, pH7.3. |
| Isotype: | IgG |
| Sequence: | Email for sequence |
| Gene ID: | 875 |
| Uniprot: | P35520 |
| Cellular Location: | |
| Calculated MW: | 61kDa |
| Observed MW: | 61KDa |
 | Western blot - CBS Rabbit mAb (CAB11612) |
| Synonyms: | HIP4 |
| Background: | 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. This gene is a major contributor to cellular hydrogen sulfide production. Multiple alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Feb 2016] |
| UniProt Protein Function: | 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. |
| UniProt Protein Details: | Protein type:Other Amino Acids Metabolism - selenoamino acid; EC 4.2.1.22; 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; cytosol; nucleus 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 |
| NCBI Summary: | 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 Code: | P35520 |
| NCBI GenInfo Identifier: | 543959 |
| NCBI Gene ID: | 875 |
| NCBI Accession: | P35520.2 |
| UniProt Secondary Accession: | P35520,Q99425, Q9BWC5, B2R993, D3DSK4, |
| UniProt Related Accession: | P35520 |
| Molecular Weight: | 61,863 Da |
| NCBI Full Name: | Cystathionine beta-synthase |
| NCBI Synonym Full Names: | cystathionine-beta-synthase |
| NCBI Official Symbol: | CBS |
| NCBI Official Synonym Symbols: | HIP4 |
| NCBI Protein Information: | cystathionine beta-synthase; beta-thionase; serine sulfhydrase; methylcysteine synthase |
| UniProt Protein Name: | Cystathionine beta-synthase |
| UniProt Synonym Protein Names: | Beta-thionase; Serine sulfhydrase |
| Protein Family: | Cystathionine beta-synthase |
| UniProt Gene Name: | CBS |
| UniProt Entry Name: | CBS_HUMAN |
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