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羥輔酶A脫氫酶β抗體
  • 產(chǎn)品貨號(hào)：
    BN41037R
  • 中文名稱(chēng)：
    羥輔酶A脫氫酶β抗體
  • 英文名稱(chēng)：
    Rabbit anti-HADHB Polyclonal antibody
  • 品牌：
    Biorigin

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  • BN41037R-100ul

    100ul

    ￥2360.00

    交叉反應(yīng)：Human,Mouse(predicted:Rat,Dog,Pig,Cow,Rabbit,Sheep) 推薦應(yīng)用：WB,IHC-P,IHC-F,IF,ELISA

  • BN41037R-200ul

    200ul

    ￥3490.00

    交叉反應(yīng)：Human,Mouse(predicted:Rat,Dog,Pig,Cow,Rabbit,Sheep) 推薦應(yīng)用：WB,IHC-P,IHC-F,IF,ELISA

產(chǎn)品描述

英文名稱(chēng)HADHB
中文名稱(chēng)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(lǐng)域腫瘤  細(xì)胞生物  免疫學(xué)  轉(zhuǎn)錄調(diào)節(jié)因子  激酶和磷酸酶  
抗體來(lái)源Rabbit
克隆類(lèi)型Polyclonal
交叉反應(yīng)Human, Mouse,  (predicted: Rat, Dog, Pig, Cow, Rabbit, Sheep, )
產(chǎn)品應(yīng)用WB=1:500-2000 ELISA=1:5000-10000 IHC-P=1:100-500 IHC-F=1:100-500 IF=1:100-500 （石蠟切片需做抗原修復(fù)）
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量47kDa
細(xì)胞定位細(xì)胞漿 細(xì)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
儲(chǔ) 存 液0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存條件Shipped at 4℃. Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.
PubMedPubMed
產(chǎn)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



Important Note:
This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications.















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