丙酮酸脱氢酶α1抗体
规格：1mg/1ml
英文名： PDHA1
别名： mitochondrial; somatic form; ODPA_HUMAN; PDH; PDHA1; PDHCE1A; PDHE1 A type I; PDHE1-A type I; PHE1A; Pyruvate Dehydrogenase (lipoamide) alpha 1; Pyruvate Dehydrogenase E1 alpha; Pyruvate dehydrogenase
分子量： 43kDa
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 PDHA1
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Rabbit,
细胞定位：细胞浆 线粒体
丙酮酸脱氢酶α1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 CO(2), 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 alpha 1 subunit containing the E1 active site, and plays a key role in the function of the PDH complex. Mutations in this gene are associated with pyruvate dehydrogenase E1-alpha deficiency and X-linked Leigh syndrome. Alternatively spliced transcript variants encoding different isoforms have been found for this gene. Function: The pyruvate dehydrogenase complex catalyzes the overall conversion of pyruvate to acetyl-CoA and CO(2), and thereby links the glycolytic pathway to the tricarboxylic cycle. Subunit: Tetramer of 2 alpha and 2 beta subunits. Subcellular Location: Mitochondrion matrix. Tissue Specificity: Testis. Expressed in postmeiotic spermatogenic cells. Post-translational modifications: Phosphorylation at Ser-232, Ser-293 and Ser-300 by PDK family kinases inactivates the enzyme; for this phosphorylation at a single site is sufficient. Dephosphorylation at all three sites, i.e. at Ser-232, Ser-293 and Ser-300, is required for reactivation. DISEASE: Pyruvate dehydrogenase E1-alpha deficiency 丙酮酸脱氢酶α1抗体(PDHAD) [MIM:312170]: 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. Note=The disease is caused by mutations affecting the gene represented in this entry. X-linked Leigh syndrome (X-LS) [MIM:308930]: Early-onset progressive neurodegenerative disorder with a characteristic neuropathology consisting of focal, bilateral lesions in one or more areas of the central nervous system, including the brainstem, thalamus, basal ganglia, cerebellum, and spinal cord. The lesions are areas of demyelination, gliosis, necrosis, spongiosis, or capillary proliferation. Clinical symptoms depend on which areas of the central nervous system丙酮酸脱氢酶α1抗体 are involved. The most common underlying cause is a defect in oxidative phosphorylation. LS may be a feature of a deficiency of any of the mitochondrial respiratory chain complexes. Note=The disease is caused by mutations affecting the gene represented in this entry. Database links: Entrez Gene: 407109 Cow Entrez Gene: 5160 Human Entrez Gene: 18597 Mouse Entrez Gene: 29554 Rat Omim: 300502 Human SwissProt: A7MB35 Cow SwissProt: P08559 Human SwissProt: P35486 Mouse SwissProt: P26284 Rat Unigene: 530331 Human Unigene: 34775 Mouse Unigene: 3655 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α1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 **学 激酶和磷酸酶 线粒体
储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
来源： Rabbit
外观： Lyophilized or Liquid