丙酮酸脱氢酶E1β亚单位重组兔单抗

Rrmab®兔单抗
2026-01-04~2026-02-28,RR26012026-01-04~2026-02-28,TR
丙酮酸脱氢酶E1β亚单位重组兔单抗
货号:bsm-61702R
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概述

产品编号
bsm-61702R
产品类型
重组兔单抗、mIHC精品抗体
英文名称
PDHB Recombinant Rabbit mAb
中文名称
丙酮酸脱氢酶E1β亚单位重组兔单抗
英文别名
E1beta; PDHBD; PDHE1-B; PDHE1B; PHE1B; 2610103L06Rik; ODPB_HUMAN; PDHB; 1.2.4.1; ODPB_MOUSE; ODPB_RAT;
抗体来源
Rabbit
免疫原
A synthesized peptide derived from human PDHB: 300-359/359
亚型
IgG
性状
Liquid
纯化方法
affinity purified by Protein A
克隆类型
Recombinant
克隆号
4H1
理论分子量
39
检测分子量
35
储存液
10mM phosphate buffered saline(pH 7.4) with 150mM sodium chloride, 0.05% BSA, 0.02% Proclin300 and 50% glycerol.
SWISS
Gene ID
保存条件
Store at 4℃ for short term. Store at -20℃ for long term. 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
The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO2, and thereby links the glycolytic pathway to the tricarboxylic cycle.
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产品应用

应用已检合格种属预测种属推荐稀释比例
WBHuman, Mouse, Rat1:500-2000
IHC-PHuman, Mouse, Rat1:100-500
IHC-FHuman, Mouse, Rat1:100-500
IFHuman, Mouse, Rat1:100-500
Flow-CytHuman, Mouse, Rat1:50-100
ICC/IFHuman, Mouse, Rat1:50-200
IPHuman, Mouse, Rat1:20-50

交叉反应

交叉反应: Human, Mouse, Rat

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靶标

基因名
PDHB
蛋白名
Pyruvate dehydrogenase E1 component subunit beta, mitochondrial
亚基
Tetramer of 2 alpha and 2 beta subunits.
亚细胞定位
Mitochondrion matrix.
疾病
Defects in PDHB are the cause of pyruvate dehydrogenase E1-beta deficiency (PDHBD) [MIM:614111]. An enzymatic defect causing primary lactic acidosis in children. It is associated with a broad clinical spectrum ranging from fatal lactic acidosis in the newborn to chronic neurologic dysfunction with structural abnormalities in the central nervous system without systemic acidosis.
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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