人 Pyruvate Dehydrogenase E2 抗体对
01AB02157
Dihydrolipoamide S-acetyltransferase (DLAT) is the E2 core enzyme of the mitochondrial pyruvate dehydrogenase complex, essential for cellular energy metabolism by catalyzing the transfer of acetyl groups from pyruvate to coenzyme A, thus linking glycolysis to the tricarboxylic acid (TCA) cycle. Structurally, DLAT is a multidomain protein with three tandem lipoyl domains, a peripheral subunit binding domain, and a catalytic domain, assembling into a multimeric core that anchors the complex and facilitates substrate channeling. DLAT is also the primary mitochondrial autoantigen in primary biliary cirrhosis (PBC), where antimitochondrial antibodies target the protein, leading to immune-mediated destruction of bile duct epithelial cells, progressive cholestasis, and ultimately cirrhosis and liver failure. Additionally, mutations in DLAT cause pyruvate dehydrogenase E2 deficiency, resulting in impaired pyruvate metabolism, lactic acidosis, severe neurological deficits, and developmental delay, particularly affecting infants and young children due to the brain¡¯s high energy demands.
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技术参数
- 靶点基因名
- DLAT
- 基因别名
- DLTA
- 蛋白别名
- dihydrolipoamide S-acetyltransferase; Pyruvate Dehydrogenase E2
- UniProt
- P10515
- 物种
- Human
产品详情
- 靶点名称
- Pyruvate Dehydrogenase E2
- 蛋白名称
- dihydrolipoamide S-acetyltransferase
- 蛋白别名
- Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex; mitochondrial; 70 kDa mitochondrial autoantigen of primary biliary cirrhosis; PBC; Dihydrolipoamide acetyltransferase component of pyruvate dehydrogenase complex; M2 antigen complex 70 kDa subunit; Pyruvate dehydrogenase complex component E2; PDC-E2; PDCE2
- 靶点基因名
- DLAT
- 基因别名
- DLTA
- UniProt
- P10515
- 来源物种
- Homo sapiens (Human)
- 物种反应性
- Human
背景介绍
Dihydrolipoamide S-acetyltransferase (DLAT) is the E2 core enzyme of the mitochondrial pyruvate dehydrogenase complex, essential for cellular energy metabolism by catalyzing the transfer of acetyl groups from pyruvate to coenzyme A, thus linking glycolysis to the tricarboxylic acid (TCA) cycle. Structurally, DLAT is a multidomain protein with three tandem lipoyl domains, a peripheral subunit binding domain, and a catalytic domain, assembling into a multimeric core that anchors the complex and facilitates substrate channeling. DLAT is also the primary mitochondrial autoantigen in primary biliary cirrhosis (PBC), where antimitochondrial antibodies target the protein, leading to immune-mediated destruction of bile duct epithelial cells, progressive cholestasis, and ultimately cirrhosis and liver failure. Additionally, mutations in DLAT cause pyruvate dehydrogenase E2 deficiency, resulting in impaired pyruvate metabolism, lactic acidosis, severe neurological deficits, and developmental delay, particularly affecting infants and young children due to the brain¡¯s high energy demands.