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DLAT Antibody

CAT: 0247-OAGA03468-100ULSize: 100 µLDry Ice: NoHazardous: No
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CAT#:0247-OAGA03468-100ULSize:100 µL
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CAS Number
9007-83-4
Gene Name
Dihydrolipoamide S-acetyltransferase
Gene Aliases
E2, PBC, DLTA, PDCE2, PDC-E2
Gene ID
1737
Accession Number
NP_001922.2
Reactivity
Human
Immunogen
Recombinant protein encompassing a sequence within the center region of human DLAT. The exact sequence is proprietary.
Target
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.
Clonality
Polyclonal
Type
Polyclonal Antibody
Applications
IHC-P, WB
Purification
Purified by antigen-affinity chromatography.
Assay Protocol
Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Reconstitution & Storage Instructions Reconstitution & Storage Instructions Western Blotting/Immunoblotting (WB/IB) Protocol Western Blotting/Immunoblotting (WB/IB) Protocol Immunohistochemistry (IHC) Protocol Immunohistochemistry (IHC) Protocol Immunocytochemistry (ICC) Protocol Immunocytochemistry (ICC) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Enzyme-Linked ImmunoSorbent Assay (ELISA) Protocol Blocking Peptide Competition Protocol (BPCP) Blocking Peptide Competition Protocol (BPCP) Immunoprecipitation (IP) Protocol Immunoprecipitation (IP) Protocol Antibody Array (AA) Protocol Antibody Array (AA) Protocol
Concentration
1 mg/ml
Format
Liquid
Buffer
1XPBS, 1% BSA, 20% Glycerol (pH7) . 0.01% Thimerosal was added as a preservative.
Reconstitution
Keep as concentrated solution. Aliquot and store at -20C or below. Avoid multiple freeze-thaw cycles.
Fragment
IgG
NCBI Gene Symbol
DLAT
Host or Source
Rabbit
Protein Name
Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
Gene Name URL
DLAT
Nucleotide Accession Number
NM_001931.4

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