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Mouse Anti-DLAT Recombinant Antibody (15B143) (CBMAB-D1006-YC)


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Summary

Host Animal
Mouse
Specificity
Human, Mouse
Clone
15B143
Antibody Isotype
IgG
Application
IIHC-Fr, IP, WB

Basic Information

Immunogen
Recombinant full length Human DLAT
Specificity
Human, Mouse
Antibody Isotype
IgG
Clonality
Monoclonal
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Storage
Store at 4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freeze/thaw cycles.

Target

Full Name
Dihydrolipoamide S-Acetyltransferase
Introduction
DLAT is a 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.
Entrez Gene ID
Human1737
Mouse235339
UniProt ID
HumanP10515
MouseQ8BMF4
Alternative Names
Dihydrolipoamide S-Acetyltransferase; Dihydrolipoamide Acetyltransferase Component Of Pyruvate Dehydrogenase Complex; 70 KDa Mitochondrial Autoantigen Of Primary Biliary Cirrhosis; E2 Component Of Pyruvate Dehydrogenase Complex; Pyruvate Dehydrogenase Complex Component E2; M2 Antigen Complex 70 KDa Subunit; EC 2.3.1.12;
Function
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2, and thereby links the glycolytic pathway to the tricarboxylic cycle.
Biological Process
Acetyl-CoA biosynthetic process from pyruvate Source: GO_Central
Glucose metabolic process Source: UniProtKB-KW
Sleep Source: Ensembl
Tricarboxylic acid cycle Source: UniProtKB-KW
Cellular Location
Mitochondrion matrix
Involvement in disease
Primary biliary cirrhosis is a chronic, progressive cholestatic liver disease characterized by the presence of antimitochondrial autoantibodies in patients' serum. It manifests with inflammatory obliteration of intra-hepatic bile duct, leading to liver cell damage and cirrhosis. Patients with primary biliary cirrhosis show autoantibodies against the E2 component of pyruvate dehydrogenase complex.
Pyruvate dehydrogenase E2 deficiency (PDHE2 deficiency):
Pyruvate dehydrogenase (PDH) deficiency is a major cause of primary lactic acidosis and neurological dysfunction in infancy and early childhood. In this form of PDH deficiency episodic dystonia is the major neurological manifestation, with other more common features of pyruvate dehydrogenase deficiency, such as hypotonia and ataxia, being less prominent.
PTM
Delipoylated at Lys-132 and Lys-259 by SIRT4, delipoylation decreases the PHD complex activity.
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For research use only. Not intended for any clinical use.

Custom Antibody Labeling

We also offer labeled antibodies developed using our catalog antibody products and nonfluorescent conjugates (HRP, AP, Biotin, etc.) or fluorescent conjugates (Alexa Fluor, FITC, TRITC, Rhodamine, Texas Red, R-PE, APC, Qdot Probes, Pacific Dyes, etc.).

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