Rabbit anti-Human DLAT Polyclonal Antibody | anti-DLAT antibody

DLAT (Dihydrolipoyllysine-residue Acetyltransferase Component of Pyruvate Dehydrogenase Complex, Mitochondrial, 70kD Mitochondrial Autoantigen of Primary Biliary Cirrhosis, PBC, Dihydrolipoamide Acetyltransferase Component of Pyruvate Dehydrogenase Comple

Cat. Num. AAA6376261

• Gene Names: DLAT; DLTA; PDCE2; PDC-E2
• Reactivity: Human
• Applications: Western Blot
• Purity: Purified by Protein A Affinity Chromatography.
• Synonyms: DLAT; Polyclonal Antibody; DLAT (Dihydrolipoyllysine-residue Acetyltransferase Component of Pyruvate Dehydrogenase Complex; Mitochondrial; 70kD Mitochondrial Autoantigen of Primary Biliary Cirrhosis; PBC; Dihydrolipoamide Acetyltransferase Component of Pyruvate Dehydrogenase Comple; anti-DLAT antibody

• Host: Rabbit
• Reactivity: Human
• Clonality: Polyclonal
• Isotype: IgG
• Specificity: Recognizes human DLAT.
• Purity/Purification: Purified by Protein A Affinity Chromatography.
• Form/Format: Supplied as a liquid in PBS, pH 7.2. No preservative added. Labeled with Allophycocyanin (APC).

• Applicable Applications for anti-DLAT antibody: Western Blot (WB)
• Application Notes: Applications are based on unconjugated antibody.
• Immunogen: Full length human DLAT, aa1-647 (NP_001922.2).
• Immunogen Sequence: MWRVCARRAQNVAPWAGLEARWTALQEVPGTPRVTSRSGPAPARRNSVTTGYGGVRALCGWTPSSGATPRNRLLLQLLGSPGRRYYSLPPHQKVPLPSLSPTMQAGTIARWEKKEGDKINEGDLIAEVETDKATVGFESLEECYMAKILVAEGTRDVPIGAIICITVGKPEDIEAFKNYTLDSSAAPTPQAAPAPTPAATASPPTPSAQAPGSSYPPHMQVLLPALSPTMTMGTVQRWEKKVGEKLSEGDLLAEIETDKATIGFEVQEEGYLAKILVPEGTRDVPLGTPLCIIVEKEADISAFADYRPTEVTDLKPQVPPPTPPPVAAVPPTPQPLAPTPSAPCPATPAGPKGRVFVSPLAKKLAVEKGIDLTQVKGTGPDGRITKKDIDSFVPSKVAPAPAAVVPPTGPGMAPVPTGVFTDIPISNIRRVIAQRLMQSKQTIPHYYLSIDVNMGEVLLVRKELNKILEGRSKISVNDFIIKASALACLKVPEANSSWMDTVIRQNHVVDVSVAVSTPAGLITPIVFNAHIKGVETIANDVVSLATKAREGKLQPHEFQGGTFTISNLGMFGIKNFSAIINPPQACILAIGASEDKLVPADNEKGFDVASMMSVTLSCDHRVVDGAVGAQWLAEFRKYLEKPITMLL
• Conjugate: APC
• Note: Preservative Free
• Preparation and Storage: Store product at 4 degree C in the dark. DO NOT FREEZE! Stable at 4 degree C for 12 months after receipt as an undiluted liquid. Dilute required amount only prior to immediate use. Further dilutions can be made in assay buffer. Caution: APC conjugates are sensitive to light. For maximum recovery of product, centrifuge the original vial prior to removing the cap.

Western Blot (WB)

(DLAT rabbit polyclonal antibody. Western Blot analysis of DLAT expression in human pancreas.)

Western Blot (WB)

(DLAT rabbit polyclonal antibody. Western Blot analysis of DLAT expression in A-431.)

Western Blot (WB)

(Western Blot analysis of DLAT expression in transfected 293T cell line by DLAT polyclonal antibody. Lane 1: DLAT transfected lysate (69kD). Lane 2: Non-transfected lysate.)

Product Categories/Family for anti-DLAT antibody

Antibodies; Enzymes; Dehydrogenase

NCBI and Uniprot Product Information

• NCBI GI #: 31711992
• NCBI GeneID: 1737
• NCBI Accession #: NP_001922.2
• NCBI GenBank Nucleotide #: NM_001931.2
• UniProt Accession #: P10515; Q16783; Q53EP3
• Molecular Weight: 68,997 Da
• NCBI Official Full Name: dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
• NCBI Official 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; 70 kDa mitochondrial autoantigen of primary biliary cirrhosis; E2 component of pyruvate dehydrogenase complex; M2 antigen complex 70 kDa subunit; PBC
• UniProt Protein Name: Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
• Protein Family: Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex
• UniProt Gene Name: DLAT
• UniProt Synonym Gene Names: DLTA; PBC; PDC-E2; PDCE2
• UniProt Entry Name: ODP2_HUMAN

NCBI Description

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 Description

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.

Protein type: Carbohydrate Metabolism - pyruvate; Carbohydrate Metabolism - glycolysis and gluconeogenesis; EC 2.3.1.12; Transferase; Carbohydrate Metabolism - citrate (TCA) cycle; Mitochondrial

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