内质网Aβ相关结合蛋白抗体

内质网Aβ相关结合蛋白抗体

规格：1mg/1ml

英文名： ERAB/HSD17B10

别名： HSD17B10; Mitochondrial L3 Hydroxyacyl CoA Dehydrogenase; 17 beta hydroxysteroid dehydrogenase 10; 17 beta hydroxysteroid dehydrogenase type 10; 17b HSD10; 3 hydroxy 2 methylbutyryl CoA dehydrogenase;

分子量： 21kDa

储存液：0.01M TBS(pH7.4) with 1% BSA, 0.03% Proclin300 and 50% Glyce

克隆类型：Polyclonal

亚型：IgG

纯化方法：affinity purified by Protein A

**原：KLH conjugated synthetic peptide derived from human ERAB

交叉反应：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, Guinea Pig,

内质网Aβ相关结合蛋白抗体细胞定位：细胞浆 线粒体

产品介绍：background: This gene encodes 3-hydroxyacyl-CoA dehydrogenase type II, a member of the short-chain dehydrogenase/reductase superfamily. The gene product is a mitochondrial protein that catalyzes the oxidation of a wide variety of fatty acids, alcohols, and steroids. The protein 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). Several alternatively spliced transcript variants have been identified, but the full-length nature of only two transcript variants has been determined. [provided by RefSeq, Jul 2008]. 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.内质网Aβ相关结合蛋白抗体 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: 2-methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency (MHBD deficiency) [MIM:300438]: A disorder that leads to neurological abnormalities, including psychomotor retardation and, in virtually all patients, loss of mental and motor skills. Note=The disease is caused by mutations affecting the gene represented in this entry. Mental retardation, X-linked, syndromic, 10 (MRXS10) [MIM:300220]: A disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptative behavior and manifested during the developmental period. 内质网Aβ相关结合蛋白抗体MRXS10 patients manifest mild mental retardation, choreoathetosis and abnormal behavior. Note=The disease is caused by mutations affecting the gene represented in this entry. Mental retardation, X-linked 17 (MRX17) [MIM:300705]: A disorder characterized by significantly below average general intellectual functioning associated with impairments in adaptative behavior and manifested during the developmental period. Intellectual deficiency is the only primary symptom of non-syndromic X-linked mental retardation, while syndromic mental retardation presents with associated physical, neurological and/or psychiatric manifestations. Note=The gene represented in this entry is involved in disease pathogenesis. A chromosomal microduplication involving HSD17B10 and HUWE1 has been found in patients with mental retardation. Similarity: Belongs to the short-chain dehydrogenases/reductases (SDR) family. Gene ID: 3028 Database links: Entrez Gene: 3028 Human Entrez Gene: 15108 Mouse Entrez Gene: 63864 Rat Omim: 300256 Human SwissProt: Q99714 Human SwissProt: O08756 Mouse SwissProt: O70351 Rat Unigene: 171280 Human Unigene: 6994 Mouse Unigene: 2700 Rat Important Note: This product as supplied is intended for research use only, not for use in human, therapeutic or diagnostic applications. 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。

内质网Aβ相关结合蛋白抗体产品应用：ELISA=1:500-1000 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.

研究领域：**学  神经生物学  线粒体  

储存条件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

来源： Mouse

外观： Lyophilized or Liquid

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