丙酰辅酶A羧化酶β链抗体
规格：1mg/1ml
英文名： PCCB
别名： DKFZp451E113; PCCase subunit beta; pccB; PCCB_HUMAN; pccBC Complementation group; Propanoyl CoA:carbon dioxide ligase subunit beta; Propanoyl-CoA:carbon dioxide ligase subunit beta; Propionyl CoA carb
分子量： 58kDa
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 PCCB
交叉反应：Human, Mouse, Rat, Chicken, Dog, Pig, Cow, Horse, Sheep,
细胞定位：
丙酰辅酶A羧化酶β链抗体产品介绍：background: The protein encoded by this gene is a subunit of the propionyl-CoA carboxylase (PCC) enzyme, which is involved in the catabolism of propionyl-CoA. PCC is a mitochondrial enzyme that probably acts as a dodecamer of six alpha subunits and six beta subunits. This gene encodes the beta subunit of PCC. Defects in this gene are a cause of propionic acidemia type II (PA-2). 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 PCCB are the cause of propionic acidemia type II (PA-2) [MIM:606054]. PA-2 is a life-threatening disease characterized by episodic vomiting丙酰辅酶A羧化酶β链抗体, lethargy and ketosis, neutropenia, periodic thrombocytopenia, hypogammaglobulinemia, developmental retardation, and intolerance to protein. Similarity: Belongs to the AccD/PCCB family. Contains 1 carboxyltransferase domain. Gene ID: 5096 Database links: Entrez Gene: 5096 Human Entrez Gene: 66904 Mouse Entrez Gene: 24624 Rat Omim: 232050 Human SwissProt: Q2TBR0 Cow SwissProt: 丙酰辅酶A羧化酶β链抗体P05166 Human SwissProt: Q99MN9 Mouse SwissProt: P79384 Pig SwissProt: P07633 Rat Unigene: 63788 Human Unigene: 335385 Mouse Unigene: 9732 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β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：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