Human DLAT Recombinant Protein (RPPB1577)
- SKU:
- RPPB1577
- Product type:
- Recombinant Protein
- Size:
- 10ug
- Species:
- Human
- Target:
- DLAT
- Synonyms:
- Dihydrolipoamide S-Acetyltransferase
- PDC-E2
- dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial
- Pyruvate dehydrogenase complex component E2
- Source:
- Sf9 Insect cells
- Uniprot:
- P10515
Description
| Product Name: | Human DLAT Recombinant Protein |
| Product Code: | RPPB1577 |
| Size: | 10µg |
| Species: | Human |
| Target: | DLAT |
| Synonyms: | Dihydrolipoamide S-Acetyltransferase, PDC-E2, dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial, Pyruvate dehydrogenase complex component E2, 70 kDa mitochondrial autoantigen of primary biliary cirrhosis, DLAT, PBC, M2 antigen complex 70 kDa subunit, EC 2.3.1.12, EC 2.3.1. |
| Source: | Sf9 Insect cells |
| Physical Appearance: | Sterile Filtered colorless solution. |
| Formulation: | DLAT (0.91mg/ml) is supplied in 20mM HEPES buffer pH-8.0, 200mM NaCl and 20% glycerol. |
| Stability: | Store DLAT at 4°C if entire vial will be used within 2-4 weeks. Store, frozen at -20°C for longer periods of time. Avoid multiple freeze-thaw cycles. |
| Purity: | DLAT purity was found to be greater than 75% as determined by SDS-PAGE. |
DLAT gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC is located in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, takes acetyl groups created by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the antigen for antimitochondrial antibodies which are found in about 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In patients who suffer from this illness, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC ultimately leads to cirrhosis and liver failure. Mutations in DLAT are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lactic acidosis in infancy and early childhood.
DLAT is a full-length cDNA coding for the mature form of the human PDC-E2 protein having a molecular mass of 60,630 Dalton (pH 5.8). DLAT protein is fused to a hexa-histidine purification tag.
| UniProt Protein Function: | DLAT: the E2 component of the pyruvate dehydrogenase complex (PDHC) that catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2. Contains two lipoyl-binding domains. The PDHC plays a major role in controlling the balance between lipid and glucose oxidation depending on substrate availability. The activity of the PDHC is tightly regulated by phosphorylation of the E1 components by the PDHKs. 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 homo-dimers of dihydrolipoamide dehydrogenase (E3), plus E3 binding proteins. PDHKs are recruited to the PDHC by binding to a lipoyl group covalently attached to K174 of the inner lipoyl domain of the E2 component. |
| UniProt Protein Details: | Protein type:Mitochondrial; EC 2.3.1.12; Transferase; Carbohydrate Metabolism - citrate (TCA) cycle; Carbohydrate Metabolism - pyruvate; Carbohydrate Metabolism - glycolysis and gluconeogenesis Chromosomal Location of Human Ortholog: 11q23.1 Cellular Component: mitochondrion; mitochondrial matrix; mitochondrial pyruvate dehydrogenase complex Molecular Function:dihydrolipoyllysine-residue acetyltransferase activity; protein binding Biological Process: cellular metabolic process; tricarboxylic acid cycle; glucose metabolic process; regulation of acetyl-CoA biosynthetic process from pyruvate; pyruvate metabolic process Disease: Pyruvate Dehydrogenase E2 Deficiency |
| NCBI Summary: | This gene encodes component E2 of the multi-enzyme pyruvate dehydrogenase complex (PDC). PDC resides in the inner mitochondrial membrane and catalyzes the conversion of pyruvate to acetyl coenzyme A. The protein product of this gene, dihydrolipoamide acetyltransferase, accepts acetyl groups formed by the oxidative decarboxylation of pyruvate and transfers them to coenzyme A. Dihydrolipoamide acetyltransferase is the antigen for antimitochondrial antibodies. These autoantibodies are present in nearly 95% of patients with the autoimmune liver disease primary biliary cirrhosis (PBC). In PBC, activated T lymphocytes attack and destroy epithelial cells in the bile duct where this protein is abnormally distributed and overexpressed. PBC enventually leads to cirrhosis and liver failure. Mutations in this gene are also a cause of pyruvate dehydrogenase E2 deficiency which causes primary lactic acidosis in infancy and early childhood.[provided by RefSeq, Oct 2009] |
| UniProt Code: | P10515 |
| NCBI GenInfo Identifier: | 215274207 |
| NCBI Gene ID: | 1737 |
| NCBI Accession: | P10515.3 |
| UniProt Secondary Accession: | P10515,Q16783, Q53EP3, |
| UniProt Related Accession: | P10515 |
| Molecular Weight: | 647 |
| NCBI Full Name: | Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial |
| NCBI Synonym Full Names: | dihydrolipoamide S-acetyltransferase |
| NCBI Official Symbol: | DLAT |
| NCBI Official Synonym Symbols: | DLTA; PDCE2; PDC-E2 |
| NCBI Protein Information: | dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial; PBC; M2 antigen complex 70 kDa subunit; pyruvate dehydrogenase complex component E2; E2 component of pyruvate dehydrogenase complex; 70 kDa mitochondrial autoantigen of primary biliary cirrhosis; dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial |
| UniProt Protein Name: | Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial |
| UniProt Synonym Protein Names: | 70 kDa mitochondrial autoantigen of primary biliary cirrhosis; PBC; Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; M2 antigen complex 70 kDa subunit; Pyruvate dehydrogenase complex component E2; PDC-E2; PDCE2 |
| Protein Family: | Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex |
| UniProt Gene Name: | DLAT |
| UniProt Entry Name: | ODP2_HUMAN |
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