内质网Aβ相关结合蛋白抗体

英文名称  Anti-ERAB/HSD17B10 

中文名称  内质网Aβ相关结合蛋白抗体 

内质网Aβ相关结合蛋白抗体别    名  Mitochondrial L3 Hydroxyacyl CoA Dehydrogenase; 17 beta hydroxysteroid dehydrogenase 10; 17 beta hydroxysteroid dehydrogenase type 10; 17b HSD10; 3 hydroxy 2 methylbutyryl CoA dehydrogenase; 3 hydroxyacyl CoA dehydrogenase type 2; 3 hydroxyacyl CoA dehydrogenase type II; AB binding alcohol dehydrogenase; ABAD; Ads9; Amyloid beta binding polypeptide; Amyloid beta peptide binding alcohol dehydrogenase; Amyloid beta peptide binding protein; CAMR; DUPXp11.22; Endoplasmic Reticulum Amyloid Binding Protein; Endoplasmic reticulum associated amyloid beta peptide binding protein; ER associated amyloid beta-binding protein; ERAB; HADH 2; HADH2; HCD 2; HCD2; HSD17B10; Hydroxyacyl CoA Dehydrogenase type II; Hydroxyacyl Coenzyme A dehydrogenase type II; Hydroxysteroid (17 beta) dehydrogenase 10; Mental retardation X linked syndromic 11; MHBD; Mitochondrial L3 Hydroxyacyl CoA Dehydrogenase; Mitochondrial ribonuclease P protein 2; Mitochondrial RNase P protein 2; MRPP2; MRX17; SCHAD; SDR5C1; Short chain dehydrogenase/reductase family 5C member 1; Short chain L 3 hydroxyacyl CoA dehydrogenase type 2; Short chain type dehydrogenase/reductase XH98G2; Type 10 17b HSD; Type 10 17beta hydroxysteroid dehydrogenase; Type II HADH; XH98G2. 

浓    度  1mg/1ml 

规 格  0.1ml/100μg  0.2ml/200μg     

抗体来源  Mouse  

克隆类型  polyclonal 

交叉反应  Human, Mouse, Rat

产品类型  一抗    

公司全程供应Abcam、CST、Abnova、BD、GeneTex、Proteintech、SB、Abgent、AbFrontier、AnaSpec、Agrisera、Alomone、Epitomics、Active Motif、Assaybiotech、Biorbyt、BioVision、Bender、CaymanChemical、Covance、ChromoTek、Enzo、eBioscience、Glen Research、MBL、ProSci、PeproTech、Swant、Vector等品牌公司抗体，产品货期短、内质网Aβ相关结合蛋白抗体品质保证、全国免邮，并提供抗体相应实验技术指导。

研究领域  免疫学 神经生物学  

蛋白分子量  predicted molecular weight: 21kDa 

性    状  Lyophilized or Liquid 

免 疫 原  KLH conjugated synthetic peptide derived from human ERAB 

亚    型  IgG 

纯化方法  affinity purified by Protein A 

储 存 液  0.01M PBS, pH 7.4 with 10 mg/ml BSA and 0.1% Sodium azide 

产品应用   WB=1:100-500  ELISA=1:500-1000  IP=1:20-100  IHC-P=1:100-500  IHC-F=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.  

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. 

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

内质网Aβ相关结合蛋白抗体产品介绍 ERAB is a member of the short-chain dehydrogenase/reductase superfamily. This mitochondrial protein catalyzes the oxidation of a wide variety of fatty acids, alcohols, and steroids. It has been implicated in the development of Alzheimer's disease, and mutations in the gene are the cause of 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency (MHBD, a disorder characterized by neurological abnormalities, including psychomotor retardation and loss of mental and motor skills). Several alternatively spliced transcript variants have been identified, but the full-length nature of only two transcript variants has been determined. Abundant in human liver, kidney and gonads, but it is present in only negligible amounts in skeletal muscle. At the sub-cellular level, in normal tissues, this protein is located in mithocondria. It is over-expressed in Neurons of patients with Alzheimer's disease.

Function : Functions in mitochondrial tRNA maturation. Part of mitochondrial ribonuclease P, an enzyme composed of MRPP1/RG9MTD1, MRPP2/HSD17B10 and MRPP3/KIAA0391, which cleaves tRNA molecules in their 5'-ends. By interacting with intracellular amyloid-beta, it may contribute to the neuronal dysfunction associated with Alzheimer disease (AD).

Subcellular Location : Mitochondrion

Tissue Specificity : Expressed in normal tissues but is overexpressed in neurons affected in AD.

DISEASE : Defects in HSD17B10 are the cause of 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency (MHBD deficiency) [MIM:300438]. MHBD deficiency leads to neurological abnormalities, including psychomotor retardation, and, in virtually all patients, loss of mental and motor skills. 

Defects in HSD17B10 are the cause of mental retardation syndromic X-linked type 10 (MRXS10) [MIM:300220]. MRXS10 is characterized by mild mental retardation, choreoathetosis and abnormal behavior. 

Similarity : Belongs to the short-chain dehydrogenases/reductases (SDR) family.

ERAB（Endoplasmic reticulum amyloid beta-peptide binding protein）内质网Aβ相关结合蛋白是一个细胞内与Aβ结合的蛋白。Aβ是一个具有导致阿尔兹海默斯病作用的神经毒多肽。ERAB被认为是一个羟基类固醇脱氢酶。它表达在正常组织，但是，在阿尔兹海默斯病神经损伤时过渡表达，在培养细胞中当Aβ的毒性作用增加是过度表达。

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