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短鏈L-3羥烷基輔酶A脫氫酶抗體費(fèi)用 全：公司提供上萬種產(chǎn)品，涵蓋了生物試劑，elisa試劑盒，標(biāo)準(zhǔn)品，培養(yǎng)基，原裝耗材，抗體、培養(yǎng)基、ATCC細(xì)胞等，基本上各種科研所需產(chǎn)品在我司都能找到。

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短鏈L-3羥烷基輔酶A脫氫酶抗體費(fèi)用 英文名稱  Anti-SCHAD/HADHSC

中文名稱  短鏈L-3羥烷基輔酶A脫氫酶抗體費(fèi)用 

別    名 HAD; HADH; HADH1; HADHSC; HCDH; HCDH_HUMAN; HHF4; Hydroxyacyl CoA dehydrogenase; Hydroxyacyl-coenzyme A dehydrogenase; hydroxyacyl-coenzyme A dehydrogenase, mitochondrial; L 3 hydroxyacyl Coenzyme A dehydrogenase short chain; M SCHAD; Medium and short chain L 3 hydroxyacyl coenzyme A dehydrogenase; Medium and short-chain L-3-hydroxyacyl-coenzyme A dehydrogenase; MGC8392; mitochondrial; MSCHAD; OTTHUMP00000162626; OTTHUMP00000219688; SCHAD; Short chain 3 hydroxyacyl CoA dehydrogenase mitochondrial; short chain 3-hydroxyacyl-coa dehydrogenase; Short-chain 3-hydroxyacyl-CoA dehydrogenase.

產(chǎn)品屬性：

濃    度  1mg/1ml

規(guī) 格  0.2ml/200μg

抗體來源  Rabbit

克隆類型   polyclonal

交叉反應(yīng)   Human, Mouse, Rat, Pig, Cow

產(chǎn)品類型   一抗  

研究領(lǐng)域     細(xì)胞生物 信號(hào)轉(zhuǎn)導(dǎo) 激酶和磷酸酶

蛋白分子量  predicted molecular weight: 33kDa 

性    狀   Lyophilized or Liquid

免 疫 原  KLH conjugated synthetic peptide derived from human SCHAD/HADHSC

亞    型  IgG

純化方法   affinity purified by Protein A

儲(chǔ) 存 液   Preservative: 15mM Sodium Azide, Constituents: 1% BSA, 0.01M PBS, pH 7.4

短鏈L-3羥烷基輔酶A脫氫酶抗體費(fèi)用 產(chǎn)品應(yīng)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.  

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. 

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

產(chǎn)品介紹 This gene is a member of the 3-hydroxyacyl-CoA dehydrogenase gene family. The encoded protein functions in the mitochondrial matrix to catalyze the oxidation of straight-chain3-hydroxyacyl-CoAs as part of the beta-oxidation pathway. Its enzymatic activity is highest with medium-chain-length fatty acids. Mutations in this gene cause one form of familial hyperinsulinemic hypoglycemia. The human genome contains a related pseudogene of this gene on chromosome 15. [provided by RefSeq.

Function : Plays an essential role in the mitochondrial beta-oxidation of short chain fatty acids. Exerts it highest activity toward 3-hydroxybutyryl-CoA.

Subunit : Homodimer.

Subcellular Location : Mitochondrion matrix.

Tissue Specificity : Expressed in liver, kidney, pancreas, heart and skeletal muscle.

DISEASE : Defects in HADH are the cause of 3-alpha-hydroxyacyl-CoA dehydrogenase deficiency (HADH deficiency) [MIM:231530]. HADH deficiency is a metabolic disorder with various clinical presentations including hypoglycemia, hepatoencephalopathy, myopathy or cardiomyopathy, and in some cases sudden death.

Defects in HADH are the cause of familial hyperinsulinemic hypoglycemia type 4 (HHF4) [MIM:609975]; also known as persistent hyperinsulinemic hypoglycemia of infancy (PHHI) or congenital hyperinsulinism. HHF is the most common cause of persistent hypoglycemia in infancy and is due to defective negative feedback regulation of insulin secretion by low glucose levels. It causes nesidioblastosis, a diffuse abnormality of the pancreas in which there is extensive, often disorganized formation of new islets. Unless early and aggressive intervention is undertaken, brain damage from recurrent episodes of hypoglycemia may occur. HHF4 should be easily recognizable by analysis of acylcarnitine species and that this disorder responds well to treatment with diazoxide. It provides the first 'experiment of nature' that links impaired fatty acid oxidation to hyperinsulinism and that provides support for the concept that a lipid signaling pathway is implicated in the control of insulin secretion.

Similarity : Belongs to the 3-hydroxyacyl-CoA dehydrogenase family.

Database links : UniProtKB/Swiss-Prot: Q16836.3

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技術(shù)外包服務(wù):

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