SAB5702646
Anti-DLAT Antibody, clone 2Z7R10, Rabbit Monoclonal
Synonym(s):
DLTA, PDC-E2, PDCE2
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About This Item
NACRES:
NA.46
UNSPSC Code:
12352203
biological source
rabbit
conjugate
unconjugated
material
colorless
clone
2Z7R10, monoclonal
form
liquid
mol wt
70 kDa
species reactivity
rat, rat, human
concentration
0.24 mg/mL
technique(s)
western blot: 1:500 - 1:2000
color
colorless
isotype
IgG
immunogen sequence
AREGKLQPHEFQGGTFTISNLGMFGIKNFSAIINPPQACILAIGASEDKLVPADNEKGFDVASMMSVTLSCDHRVVDGAVGAQWLAEFRKYLEKPITMLL
UniProt accession no.
shipped in
wet ice
storage temp.
−20°C
target post-translational modification
unmodified
Quality Level
Gene Information
human ... DLAT(1737)
Application
WB
General 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]
Immunogen
A synthetic peptide corresponding to a sequence within amino acids 548-647 of human DLAT (P10515).
PBS with 0.02% sodium azide,0.05% BSA,50% glycerol,pH7.3.
Storage Class
10 - Combustible liquids
wgk
WGK 1
Regulatory Information
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