DLAT Recombinant Rabbit mAb (一抗) - WB,IP | Bioss

bsm-62155R

重组兔单抗

DLAT Recombinant Rabbit mAb

丙酮酸脱氢酶E2重组兔单抗

PDC-E2; PDCE2; DLTA; E2; PBC; 6332404G05Rik; noa; nrb; wu:fc14f10; wu:fc21f08; ODP2_BOVIN; DLAT; Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; Pyruvate dehydrogenase complex component E2 (PDC-E2 | PDCE2); 2.3.1.12; ODP2_HUMAN; 70 kDa mitochondrial autoantigen of primary biliary cirrhosis (PBC); M2 antigen complex 70 kDa subunit; ODP2_MOUSE; ODP2_RAT; dihydrolipoamide S-acetyltransferase; dihydrolipoyllysine-residue acetyltransferase; E2 component of pyruvate dehydrogenase complex; dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial

Rabbit

A synthesized peptide derived from human DLAT: 585-610/647

IgG

Liquid

affinity purified by Protein A

Recombinant

8C4

69 kDa

68 kDa

10mM phosphate buffered saline(pH 7.4) with 150mM sodium chloride, 0.05% BSA, 0.02% Proclin300 and 50% glycerol.

Store at 4℃ for short term. Store at -20℃ for long term. Avoid repeated freeze/thaw cycles.

This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

As part of the pyruvate dehydrogenase complex, catalyzes the transfers of an acetyl group to a lipoic acid moiety (Probable). The pyruvate dehydrogenase complex, catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2, and thereby links cytoplasmic glycolysis and the mitochondrial tricarboxylic acid (TCA) cycle.

DLAT

Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial

Mitochondrion matrix.

Note=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.
Defects in DLAT are the cause of pyruvate dehydrogenase E2 deficiency (PDHE2 deficiency) [MIM:245348]; also known as lactic acidemia due to defect of E2 lipoyl transacetylase of the pyruvate dehydrogenase complex. 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.

Belongs to the 2-oxoacid dehydrogenase family.
Contains 2 lipoyl-binding domains.

The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2). It contains multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3).