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Rabbit Anti-HADHB/BF350 Conjugated antibody (bs-5065R-BF350)
訂購熱線:400-901-9800
訂購郵箱:sales@bioss.com.cn
訂購QQ:  400-901-9800
技術支持:techsupport@bioss.com.cn
說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-5065R-BF350
英文名稱 Rabbit Anti-HADHB/BF350 Conjugated antibody
中文名稱 BF350標記的羥輔酶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.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 腫瘤  細胞生物  免疫學  轉(zhuǎn)錄調(diào)節(jié)因子  激酶和磷酸酶  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Human, Mouse, Rat, Dog, Pig, Cow, Rabbit, Sheep, )
產(chǎn)品應用 IF=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 47kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HADHB
亞    型 IgG
純化方法 affinity purified by Protein A
儲 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol
保存條件 Store at -20 °C for one year. Avoid repeated freeze/thaw cycles. The lyophilized antibody is stable at room temperature for at least one month and for greater than a year when kept at -20°C. When reconstituted in sterile pH 7.4 0.01M PBS or diluent of antibody the antibody is stable for at least two weeks at 2-4 °C.
產(chǎn)品介紹 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 (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.

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.
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