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折扣/货期| 货号：GTX49202 | 规格：100μg | 目录价：￥5300 |
产品详情
* 以下信息仅供参考，详情请以原厂网站为准
产品名称:
DLAT antibody [4A4-B6-C10]
别名:
dihydrolipoamide S-acetyltransferase , DLTA , E2 , PDC-E2 , PDCE2
反应种属:
Human, Mouse
宿主来源:
Mouse
实验应用:
ICC/IF, IP, WB
同种型:
IgG1
免疫原:
Purified recombinant human DLAT protein fragments expressed in E.coli.
克隆性:
Monoclonal
克隆号:
4A4-B6-C10
纯化方式:
Purified by affinity chromatography
偶联:
Unconjugated
产品浓度:
Batch dependent (Please refer to the vial label for the specific concentration.)
保存温度:
Store as concentrated solution. Centrifuge briefly prior to opening vial. For short-term storage (1-2 weeks), store at 4ºC. For long-term storage, aliquot and store at -20ºC or below. Avoid multiple freeze-thaw cycles.
运输温度:
4°C
预期分子量:
69
产品形式:
Liquid
存储溶液:
PBS, 0.5% BSA, 50% glycerol, 0.02% Sodium azide.
生产商:
GeneTex
功能与背景:
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]
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