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Pig Anti-DLAT Recombinant Antibody (13G2AE2BH5) (CBMAB-P0265-YC)

Provided herein is a Pig monoclonal antibody against Human Dihydrolipoamide S-Acetyltransferase. The antibody can be used for immunoassay techniques, such as WB, IHC-P, FC, ICC, IF.
See all DLAT antibodies
Published Data
Fig.2 Immunoblot analysis of mouse brain fractionation. Mouse brain homogenate was separated into heavy membrane (P7k), cytosolic (S200k) and light membrane (P200k) fractions. Distribution of mitochondrial (Mfn2, VDAC1, PDH E2), ER (Grp78), soluble (parkin) and endosomal (Rab11A) markers are shown.
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Summary

Host Animal
Pig
Specificity
Human, Mouse, Rat, Cattle
Clone
13G2AE2BH5
Antibody Isotype
IgG2a
Application
WB, IHC-P, FC, ICC, IF

Basic Information

Specificity
Human, Mouse, Rat, Cattle
Antibody Isotype
IgG2a
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 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
Rat81654
Cattle512723
UniProt ID
HumanP10515
MouseQ8BMF4
RatP08461
CattleP11180
Alternative Names
6332404G05Rik; DLTA; PDC-E2
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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