丙酰辅酶A羧化酶α链抗体
规格：1mg/1ml
英文名： PCCA
别名： mitochondrial; Pcca; pccA COMPLEMENTATION GROUP; PCCA_HUMAN; PCCase subunit alpha; Propanoyl CoA:carbon dioxide ligase subunit alpha; Propanoyl-CoA:carbon dioxide ligase subunit alpha; Propionyl CoA c
分子量： 74kDa
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 PCCA
交叉反应：Human, Mouse, Rat,
细胞定位：
丙酰辅酶A羧化酶α链抗体产品介绍：background: The protein encoded by this gene is the alpha subunit of the heterodimeric mitochondrial enzyme Propionyl-CoA carboxylase. PCCA encodes the biotin-binding region of this enzyme. Mutations in either PCCA or PCCB (encoding the beta subunit) lead to an enzyme deficiency resulting in propionic acidemia. Multiple transcript variants encoding different isoforms have been found for this gene.[provided by RefSeq, May 2010] Subunit: Probably a dodecamer composed of six biotin-containing alpha subunits and six beta subunits. Subcellular Location: Mitochondrion matrix. DISEASE: Defects in PCCA are the cause of propionic acidemia type I (PA-1) [MIM:606054]. PA-1 is a life-threatening disease characterized by episodic vomiting, lethargy and ketosis, neutropenia, periodic thrombocytopenia, 丙酰辅酶A羧化酶α链抗体hypogammaglobulinemia, developmental retardation, and intolerance to protein. Similarity: Contains 1 ATP-grasp domain. Contains 1 biotin carboxylation domain. Contains 1 biotinyl-binding domain. Gene ID: 5095 Database links: Entrez Gene: 418774 Chicken Entrez Gene: 738775 Chimpanzee Entrez Gene: 614302 Cow Entrez Gene: 476975 Dog Entrez Gene: 101127469 Gorilla Entrez Gene: 100062251 Horse Entrez 丙酰辅酶A羧化酶α链抗体Gene: 5095 Human Entrez Gene: 110821 Mouse Entrez Gene: 100456529 Orangutan Entrez Gene: 100524103 Pig Entrez Gene: 687008 Rat Entrez Gene: 699844 Rhesus monkey Entrez Gene: 437019 Zebrafish Omim: 232000 Human SwissProt: P05165 Human SwissProt: Q91ZA3 Mouse SwissProt: P14882 Rat Unigene: 80741 Human Unigene: 23876 Mouse Unigene: 6033 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
丙酰辅酶A羧化酶α链抗体产品应用：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：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