DLAT Rabbit pAb (一抗) - WB,IHC-P,IHC-F,IF | Bioss
货号：bs-19695R
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常见问题
概述
产品编号
bs-19695R
产品类型
宠物抗体、模式动物抗体、农牧业/家禽抗体
英文名称
DLAT Rabbit pAb
中文名称
丙酮酸脱氢酶E2抗体
英文别名
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
抗体来源
Rabbit
免疫原
KLH conjugated synthetic peptide derived from human PDCE2: 231-330/647
亚型
IgG
性状
Liquid
纯化方法
affinity purified by Protein A
克隆类型
Polyclonal
理论分子量
63 kDa
检测分子量
63 kDa
浓度
1mg/ml
储存液
0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
研究领域
Cancer > Cancer Metabolism > Metabolic signaling pathway > Metabolism of carbohydrates
Metabolism > Pathways and Processes > Metabolic signaling pathways > Carbohydrate metabolism
Metabolism > Pathways and Processes > Metabolic signaling pathways > Energy transfer pathways > Energy Metabolism
Metabolism > Pathways and Processes > Mitochondrial Metabolism > Mitochondrial markers
Metabolism > Types of disease > Cancer
SWISS
Gene ID
保存条件
Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事项
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
背景资料
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]
产品应用
| 应用 | 已检合格种属 | 预测种属 | 推荐稀释比例 |
|---|---|---|---|
| WB | Human, Mouse, Rat | Rabbit, Pig, Sheep, Cow, Zebrafish, Dog, Cat, GuineaPig, Horse | 1:500-2000 |
| IHC-P | Rat | Human, Mouse, Rabbit, Pig, Sheep, Cow, Zebrafish, Dog, Cat, GuineaPig, Horse | 1:100-500 |
| IHC-F | Rat | Human, Mouse, Rabbit, Pig, Sheep, Cow, Zebrafish, Dog, Cat, GuineaPig, Horse | 1:100-500 |
| IF | Rat | Human, Mouse, Rabbit, Pig, Sheep, Cow, Zebrafish, Dog, Cat, GuineaPig, Horse | 1:100-500 |
交叉反应
交叉反应: Human, Mouse, Rat (predicted: Rabbit, Pig, Sheep, Cow, Zebrafish, Dog, Cat, GuineaPig, Horse)
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靶标
基因名
DLAT
蛋白名
Dihydrolipoyllysine-residue acetyltransferase component of pyruvate dehydrogenase complex, mitochondrial
亚细胞定位
Mitochondrion matrix.
疾病
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.
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.
相似性
Belongs to the 2-oxoacid dehydrogenase family.
Contains 2 lipoyl-binding domains.
Contains 2 lipoyl-binding domains.
功能
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).
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