丙酮酸脱氢酶E1β亚基抗体
规格：1mg/1ml
英文名： PHE1B/PDH-E1 beta
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
分子量： 36kDa
储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce
克隆类型：Polyclonal
亚型：IgG
纯化方法：affinity purified by Protein A
**原：KLH conjugated synthetic peptide derived from human PHE1B/PD
交叉反应：Human, Mouse, Rat,
细胞定位：细胞浆
丙酮酸脱氢酶E1β亚基抗体产品介绍：background: 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丙酮酸脱氢酶E1β亚基抗体 (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). Gene ID: 5162 Database links: Entrez Gene: 5162 Human Entrez Gene:丙酮酸脱氢酶E1β亚基抗体 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 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
丙酮酸脱氢酶E1β亚基抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=1:100-500 ICC=1:100-500 IF=1:100-500 （石蜡切片需做抗原修复） not yet tested in other applications. optimal dilutions/concentrations should be determined by the end user.
研究领域：肿瘤 细胞生物 信号转导
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid