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Antibodies

DLAT Antibody

Product Sizes
50 ul
£186.00
CSB-PA084734-50UL
100 ul
£334.00
CSB-PA084734-100UL
About this Product
SKU:
CSB-PA084734
Additional Names:
70 kDa mitochondrial autoantigen of primary biliary cirrhosis antibody; Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex antibody; Dihydrolipoamide S Acetyltransferase antibody; Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex antibody; dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex mitochondrial antibody; DLAT antibody; DLTA antibody; E2 antibody; E2 component of pyruvate dehydrogenase complex antibody; M2 antigen complex 70 kDa subunit antibody; mitochondrial antibody; ODP2_HUMAN antibody; PBC antibody; PDC E2 antibody; PDC-E2 antibody; PDCE2 antibody; Pyruvate dehydrogenase complex component E2 antibody; Pyruvate dehydrogenase complex E2 subunit antibody
Application:
ELISA, Immunohistochemistry, Western Blot
Buffer:
PBS
Clonality:
Polyclonal
Extra Details:
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.
Host:
Rabbit
Immunogen:
Fusion protein of Human DLAT
Isotype:
IgG
Physical State:
Liquid
Purification:
Affinity Purified
Reactivities:
Human, Mouse, Rat
Shipping Conditions:
Blue Ice
Storage Conditions:
-20[o]C/-70[o]C Avoid freeze/thaw cycles.
Supplier:
Cusabio
Type:
Antibody: Polyclonal Antibody