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DLAT Monoclonal antibody

DLAT Monoclonal antibody

货号：
AM20143
规格：

20ul

50ul

100ul
价格：
￥0.00

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产品参数

别名	1F2B9, mitochondrial, DLTA, EC:2.3.1.12, ODP2, Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex,
产品类型	Monoclonal
基因名称	dihydrolipoamide S-acetyltransferase
经测试应用	WB, IHC, IF/ICC
宿主	Mouse
种属反应性	Human, Mouse, Rat
免疫原	A synthesized peptide derived from human DLAT
理论分子量	70 kDa
实际分子量	70 kDa
Gene ID(NCBI)	1737
GeneBank Accession Number	BC039084
UNIPROT	P10515
形式	Liquid
保存条件	Store at -20℃. Stable for one year after shipment. Aliquoting is unnecessary for -20℃ storage.
推荐稀释比例	1:2500-1:25000
背景信息	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.

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