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Rabbit Anti-PHE1B/PDH-E1 beta  antibody (bs-19694R)
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说明书: 50ul  100ul  200ul
50ul/1180.00元
100ul/1980.00元
200ul/2800.00元
大包装/询价
产品编号 bs-19694R
英文名称 PHE1B/PDH-E1 beta
中文名称 丙酮酸脱氢酶E1β亚基抗体
别    名 Pyruvate Dehydrogenase E1 beta subunit; PDH-E1β; DKFZp564K0164; mitochondrial; ODPB_HUMAN; pdhB; PDHBD; PDHE1 B; PDHE1-B; PHE1B; Pyruvate dehydrogenase (lipoamide) beta; Pyruvate dehydrogenase E1 beta polypeptide; Pyruvate dehydrogenase E1 component subunit beta; Pyruvate dehydrogenase E1 component subunit beta mitochondrial.  
研究领域 肿瘤  细胞生物  信号转导  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 (predicted: Human,Mouse,Rat)
产品应用 IHC-P=1:100-500, IHC-F=1:100-500, ICC=1:100-500, IF=1:100-500, ELISA=1:5000-10000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理论分子量 36kDa
细胞定位 细胞浆 
性    状 Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human PHE1B/PDH-E1 beta: 101-200/359 
亚    型 IgG
纯化方法 affinity purified by Protein A
缓 冲 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 Shipped at 4℃. Store at -20 °C 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.
PubMed PubMed
产品介绍 The pyruvate dehydrogenase (PDH) complex is a nuclear-encoded mitochondrial multienzyme complex that catalyzes the overall conversion of pyruvate to acetyl-CoA and carbon dioxide, and provides the primary link between glycolysis and the tricarboxylic acid (TCA) cycle. The PDH complex is composed of multiple copies of three enzymatic components: pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (E2) and lipoamide dehydrogenase (E3). The E1 enzyme is a heterotetramer of two alpha and two beta subunits. This gene encodes the E1 beta subunit. Mutations in this gene are associated with pyruvate dehydrogenase E1-beta deficiency. Alternatively spliced transcript variants have been found for this gene. [provided by RefSeq, Mar 2012]

Function:
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).

Subunit:
Heterotetramer of two PDHA1 and two PDHB subunits. The heterotetramer interacts with DLAT, and is part of the multimeric pyruvate dehydrogenase complex that contains multiple copies of pyruvate dehydrogenase (E1), dihydrolipoamide acetyltransferase (DLAT, E2) and lipoamide dehydrogenase (DLD, E3). These subunits are bound to an inner core composed of about 48 DLAT and 12 PDHX molecules.

Subcellular Location:
Mitochondrion matrix.

DISEASE:
Defects in PDHB are a cause of pyruvate dehydrogenase E1 component deficiency (PDHE1 deficiency) [MIM:312170]. PDHE1 deficiency is the most common enzyme defect in patients with primary lactic acidosis. It is associated with variable clinical phenotypes ranging from neonatal death to prolonged survival complicated by developmental delay, seizures, ataxia, apnea, and in some cases to an X-linked form of Leigh syndrome (LS) (Leigh encephalomyelopathy).

SWISS:
P11177

Gene ID:
5162

Database links:

Entrez Gene: 5162 Human

Entrez Gene: 68263 Mouse

Entrez Gene: 289950 Rat

Omim: 179060 Human

SwissProt: P11966 Cow

SwissProt: P11177 Human

SwissProt: Q9D051 Mouse

SwissProt: P49432 Rat

Unigene: 161357 Human

Unigene: 301527 Mouse

Unigene: 102424 Rat



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