Anti-HADHSC抗体上海钰博生物科技有限公司

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产品名称: Anti-HADHSC抗体
英文名称: HADHSC
抗体货号: YB--10020R
产品规格: 20ul/50ul/100ul/200ul
级    别: 分析纯, 见说明书
产品产地: 中国/美国
品牌商标: Ybscience
价    格: 1380元
抗原: KLH conjugated synthetic peptide derived from human HADHSC:241-314/314
抗原来源: Rabbit
抗体来源: Rabbit
适用物种: Human, Mouse, Rat, Pig, Cow,
见说明书conjugate:见说明书
Isotype:见说明书
应用范围: 科研使用
更新时间: 2018/10/11 11:28:00
详细资料:  实验方法技术资料
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使用范围:仅限科研使用,不能应用于临床
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产品详细描述

 Anti-HADHSC抗体

产品编号 YB-10020R
英文名称 HADHSC
中文名称 短链L-3羟烷基辅酶A脱氢酶抗体
别    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.  
规格价格 100ul/1380元 购买    200ul/2200元 购买    大包装/询价
说 明 书 100ul  200ul
研究领域 肿瘤  细胞生物  信号转导  激酶和***酸酶  糖尿病  新陈代谢  线粒体  
抗体来源 Rabbit
克隆类型 Polyclonal
交叉反应 Human, Mouse, Rat, Pig, Cow, 
产品应用 WB=1:500-2000 ELISA=1:500-1000 IHC-P=1:400-800 IHC-F=1:400-800 ICC=1:100-500 IF=1:100-500 (石蜡切片需做抗原修复) 
not yet tested in other applications.
optimal dilutions/concentrations should be determined by the end user.
分 子 量 33kDa
细胞定位 细胞浆 线粒体
性    状 Lyophilized or Liquid
浓    度 1mg/ml
免 疫 原 KLH conjugated synthetic peptide derived from human HADHSC:241-314/314 
亚    型 IgG
纯化方法 affinity purified by Protein A
储 存 液 0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glycerol.
保存条件 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.
PubMed PubMed
产品介绍 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.

SWISS:
Q16836

Gene ID:
3033

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. 
 
产品图片
Sample: 
Liver (Mouse) Lysate at 40 ug
Kidney (Mouse) Lysate at 40 ug
Primary: Anti- HADHSC (bs-10020R) at 1/300 dilution
Secondary: IRDye800CW Goat Anti-Rabbit IgG at 1/20000 dilution
Predicted band size: 33 kD
Observed band size: 33 kD
Sample: Liver (Mouse) Lysate at 40 ug
Primary: Anti-HADHSC (bs-10020R) at 1/300 dilution
Secondary: HRP conjugated Goat-Anti-rabbit IgG (bs-0295G-HRP) at 1/5000 dilution
Predicted band size: 33 kD
Observed band size: 33 kD
Tissue/cell: mouse fetal liver; 4% Paraformaldehyde-fixed and paraffin-embedded; 
Antigen retrieval: citrate buffer ( 0.01M, pH 6.0 ), Boiling bathing for 15min; Block endogenous peroxidase by 3% Hydrogen peroxide for 30min; Blocking buffer (normal goat serum,C-0005) at 37℃ for 20 min; 
Incubation: Anti-HADHSC Polyclonal Antibody, Unconjugated(bs-10020R) 1:200, overnight at 4°C, followed by conjugation to the secondary antibody(SP-0023) and DAB(C-0010) staining
Paraformaldehyde-fixed, paraffin embedded (Rat pancreas); Antigen retrieval by boiling in sodium citrate buffer (pH6.0) for 15min; Block endogenous peroxidase by 3% hydrogen peroxide for 20 minutes; Blocking buffer (normal goat serum) at 37°C for 30min; Antibody incubation with (HADHSC) Polyclonal Antibody, Unconjugated (bs-10020R) at 1:400 overnight at 4°C, followed by operating according to SP Kit(Rabbit) (sp-0023) instructionsand DAB staining.

生物在线声明:以上所展示的信息由企业自行提供,内容的真实性、准确性和合法性由发布企业负责。生物在线对此不承担任何保证责任。

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