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Rabbit Anti-HADHSC/HRP Conjugated antibody (bs-3661R-HRP)
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說 明 書: 100ul  
100ul/2980.00元
大包裝/詢價
產(chǎn)品編號 bs-3661R-HRP
英文名稱1 Rabbit Anti-HADHSC/HRP Conjugated antibody
中文名稱 辣根過氧化物酶標記的短鏈L-3羥烷基輔酶A脫氫酶抗體
別    名 HAD; HADH; HADH1; HADHSC; HCDH; HCDH_MOUSE; 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.  
規(guī)格價格 100ul/2980元 購買        大包裝/詢價
說 明 書 100ul  
研究領域 免疫學  轉(zhuǎn)錄調(diào)節(jié)因子  激酶和磷酸酶  糖尿病  線粒體  
抗體來源 Rabbit
克隆類型 Polyclonal
交叉反應 (predicted: Mouse, Rat, )
產(chǎn)品應用 ELISA=1:100-1000 IHC-P=1:50-200 IHC-F=1:50-200 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 35kDa
性    狀 Lyophilized or Liquid
濃    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from mouse HADHSC
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:
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:

Entrez Gene: 3033 Human

Entrez Gene: 15107 Mouse

Entrez Gene: 113965 Rat

Omim: 601609 Human

SwissProt: Q16836 Human

SwissProt: Q61425 Mouse

SwissProt: Q9WVK7 Rat

Unigene: 438289 Human

Unigene: 260164 Mouse

Unigene: 92789 Rat



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

HADHSC的缺少可導致家族性胰島素過多低血糖綜合癥。
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