Anti-PDHB Antibody (CAB4645)
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주문- SKU:
- CAB4645
- Product type:
- Antibody
- Antibody Type:
- Monoclonal Antibody
- Reactivity:
- Human
- Reactivity:
- Mouse
- Reactivity:
- Rat
- Host Species:
- Rabbit
- Isotype:
- IgG
- Synonyms:
- PDHBD
- Synonyms:
- PDHE1-B
- Synonyms:
- PHE1B
Frequently bought together:
Description
| Product Name: | PDHB Rabbit mAb |
| Product Code: | CAB4645 |
| Size: | 20uL, 50uL, 100uL |
| Synonyms: | PDHBD, PDHE1-B, PHE1B |
| Applications: | WB, IHC |
| Reactivity: | Human, Mouse, Rat |
| Host Species: | Rabbit |
| Immunogen: | A synthesized peptide derived from human Pyruvate Dehydrogenase E1 beta subunit |
| Applications: | WB, IHC |
| Recommended Dilutions: | WB 1:500 - 1:2000 IHC 1:50 - 1:200 |
| Reactivity: | Human, Mouse, Rat |
| Positive Samples: | 293T, K-562, U-251MG, Mouse liver, Mouse skeletal muscle, Mouse heart, Rat skeletal muscle, Rat kidney, Rat heart |
| Immunogen: | A synthesized peptide derived from human Pyruvate Dehydrogenase E1 beta subunit |
| Purification Method: | Affinity purification |
| Storage: | 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: | 5162 |
| Uniprot: | P11177 |
| Calculated MW: | 35kDa |
| Observed MW: | 34KDa |
| UniProt Protein Function: | PDHB: the beta subunit of pyruvate dehydrogenase (PDH), a mitochondrial matrix enzyme that catalyzes the oxidative decarboxylation of pyruvate, producing acetyl-CoA and CO2. A key enzyme in controlling the balance between lipid and glucose oxidation depending on substrate availability. The pyruvate dehydrogenase (PDH) holoenzyme is a multi-enzyme complex (PDHC) that contains 20-30 copies of pyruvate decarboxylase tetramers (2 alpha:2 beta)(E1), 60 copies of dihydrolipoamide acetyltransferase (E2), six homodimers of dihydrolipoamide dehydrogenase (E3), plus E3 binding proteins. Defects in PDHB are a cause of pyruvate dehydrogenase E1 component deficiency (PDHE1 deficiency), the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (LS). Two alternatively spliced human isoforms have been described. |
| UniProt Protein Details: | Protein type:Carbohydrate Metabolism - citrate (TCA) cycle; Amino Acid Metabolism - valine, leucine and isoleucine biosynthesis; EC 1.2.4.1; Carbohydrate Metabolism - pyruvate; Oxidoreductase; Carbohydrate Metabolism - glycolysis and gluconeogenesis; Mitochondrial; Carbohydrate Metabolism - butanoate Chromosomal Location of Human Ortholog: 3p21.1-p14.2 Cellular Component: nucleoplasm; mitochondrion; mitochondrial matrix; pyruvate dehydrogenase complex; nucleus Molecular Function:protein binding; pyruvate dehydrogenase activity; pyruvate dehydrogenase (acetyl-transferring) activity Biological Process: cellular metabolic process; acetyl-CoA biosynthetic process from pyruvate; tricarboxylic acid cycle; glucose metabolic process; regulation of acetyl-CoA biosynthetic process from pyruvate; pyruvate metabolic process Disease: Pyruvate Dehydrogenase E1-beta Deficiency |
| NCBI Summary: | The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and carbon dioxide, and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle. The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodes the E1 beta subunit. Mutations in this gene are associated with pyruvate dehydrogenase E1-beta deficiency. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Mar 2012] |
| UniProt Code: | P11177 |
| NCBI GenInfo Identifier: | 134044259 |
| NCBI Gene ID: | 5162 |
| NCBI Accession: | P11177.3 |
| UniProt Secondary Accession: | P11177,Q6FH45, Q9BQ27, Q9UFK3, B2R7L0, B4DDD7, |
| UniProt Related Accession: | P11177 |
| Molecular Weight: | 359 |
| NCBI Full Name: | Pyruvate dehydrogenase E1 component subunit beta, mitochondrial |
| NCBI Synonym Full Names: | pyruvate dehydrogenase (lipoamide) beta |
| NCBI Official Symbol: | PDHB |
| NCBI Official Synonym Symbols: | PDHBD; PHE1B; PDHE1-B |
| NCBI Protein Information: | pyruvate dehydrogenase E1 component subunit beta, mitochondrial; pyruvate dehydrogenase, E1 beta polypeptide |
| UniProt Protein Name: | Pyruvate dehydrogenase E1 component subunit beta, mitochondrial |
| Protein Family: | Pyruvate dehydrogenase E1 component |
| UniProt Gene Name: | PDHB |
| UniProt Entry Name: | ODPB_HUMAN |
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