羟辅酶A脱氢酶β抗体 HADHB

羟辅酶A脱氢酶β抗体

规格：1mg/1ml

英文名： HADHB

别名： 2 enoyl Coenzyme A (CoA) hydratase beta subunit; 3 ketoacyl Coenzyme A (CoA) thiolase of mitochondrial trifunctional protein beta subunit; 3 ketoacyl Coenzyme A thiolase; 3 ketoacyl Coenzyme A thiolas

分子量： 47kDa

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 HADHB

交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, Sheep,

细胞定位：细胞浆 细胞膜

羟辅酶A脱氢酶β抗体产品介绍：background: The HADHB gene encodes the beta subunit of the mitochondrial trifunctional protein, which catalyzes the last three steps of mitochondrial beta-oxidation of long chain fatty acids. The mitochondrial membrane-bound heterocomplex is composed of four alpha and four beta subunits, with the beta subunit catalyzing the 3-ketoacyl-CoA thiolase activity. Mutations in this gene result in trifunctional protein deficiency. The encoded protein can also bind RNA and decreases the stability of some mRNAs. The genes of the alpha and beta subunits of the mitochondrial trifunctional protein are located adjacent to each other in the human genome in a head-to-head orientation. Alternatively spliced transcript variants have been found; however, their full-length nature is not known. Subunit: Octamer of 4 alpha羟辅酶A脱氢酶β抗体 (HADHA) and 4 beta (HADHB) subunits. Interacts with RSAD2/viperin. Subcellular Location: Mitochondrion. Mitochondrion inner membrane. Mitochondrion outer membrane. Endoplasmic reticulum. DISEASE: Defects in HADHB are a cause of trifunctional protein deficiency羟辅酶A脱氢酶β抗体 (TFP deficiency) [MIM:609015]. The clinical manifestations are very variable and include hypoglycemia, cardiomyopathy and sudden death. Phenotypes with mainly hepatic and neuromyopathic involvement can also be distinguished. Biochemically, TFP deficiency is defined by the loss of all three enzyme activities of the TFP complex. Similarity: Belongs to the thiolase family. Database links: UniProtKB/Swiss-Prot: P55084.3 Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.

羟辅酶A脱氢酶β抗体产品应用：WB=1:100-500 ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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  转录调节因子  激酶和磷酸酶  

储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

来源： Rabbit

外观： Lyophilized or Liquid