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HADHB Rabbit pAb (bs-5065R)  
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產品編號 bs-5065R
英文名稱 HADHB Rabbit pAb
中文名稱 羥輔酶A脫氫酶β抗體
別    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 thiolase of mitochondrial trifunctional protein beta subunit; Acetyl CoA acyltransferase; beta ketothiolase; ECHB; HADH; Hydroxyacyl Coenzyme A(CoA) dehydrogenase beta subunit; Hydroxyacyl Coenzyme A dehydrogenase; Hydroxyacyl Coenzyme A dehydrogenase beta subunit; Hydroxyacyl Coenzyme A dehydrogenase/3 ketoacyl Coenzyme A thiolase/enoyl Coenzyme A hydratase(trifunctional protein) beta subunit; Mitochondrial trifunctional enzyme beta subunit; Mitochondrial trifunctional protein beta subunit; MTPB; TP beta. TPbeta; Trifunctional enzyme subunit beta; Trifunctional enzyme subunit beta mitochondrial; Trifunctional protein; ECHB_HUMAN.  
研究領域 腫瘤  細胞生物  免疫學  轉錄調節因子  激酶和磷酸酶  
抗體來源 Rabbit
克隆類型 Polyclonal
克 隆 號
交叉反應 Human,Mouse (predicted: Rat,Rabbit,Pig,Sheep,Cow,Dog)
產品應用 WB=1:500-2000
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
理論分子量 47 kDa
檢測分子量
細胞定位 細胞漿 細胞膜 
性    狀 Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HADHB: 231-330/474 
亞    型 IgG
純化方法 affinity purified by Protein A
緩 沖 液 0.01M TBS (pH7.4) with 1% BSA, 0.02% Proclin300 and 50% Glycerol.
保存條件 Shipped at 4℃. Store at -20℃ for one year. Avoid repeated freeze/thaw cycles.
注意事項 This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.
PubMed PubMed
產品介紹 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 (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 (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.

SWISS:
P55084

Gene ID:
3032

Database links:

Entrez Gene: 3032 Human

Omim: 143450 Human

SwissProt: P55084 Human

Unigene: 515848 Human



產品圖片
Sample: Kidney (Mouse) Lysate at 40 ug Primary: Anti- HADHB (bs-5065R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 47kD Observed band size: 47 kD
SY5Y(Human) Cell Lysate at 30 ug Primary: Anti-HADHB (bs-5065R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 47 kD Observed band size: 47 kD
Siha(Human) Cell Lysate at 30 ug Primary: Anti-HADHB (bs-5065R) at 1/1000 dilution Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution Predicted band size: 47 kD Observed band size: 47 kD
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