內(nèi)質(zhì)網(wǎng)Aβ相關(guān)結(jié)合蛋白抗體

規(guī)格：1mg/1ml

英文名： ERAB/HSD17B10

別名： 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

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

克隆類(lèi)型：Polyclonal

亞型：IgG

純化方法：affinity purified by Protein A

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

交叉反應(yīng)：Human, Mouse, Rat, Dog, Pig, Cow, Horse, Sheep, Guinea Pig,

內(nèi)質(zhì)網(wǎng)Aβ相關(guān)結(jié)合蛋白抗體細(xì)胞定位：細(xì)胞漿 線粒體

產(chǎn)品介紹：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.內(nèi)質(zhì)網(wǎng)Aβ相關(guān)結(jié)合蛋白抗體 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. 內(nèi)質(zhì)網(wǎng)Aβ相關(guān)結(jié)合蛋白抗體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）內(nèi)質(zhì)網(wǎng)Aβ相關(guān)結(jié)合蛋白是一個(gè)細(xì)胞內(nèi)與Aβ結(jié)合的蛋白。Aβ是一個(gè)具有導(dǎo)致阿爾茲海默斯病作用的神經(jīng)毒多肽。ERAB被認(rèn)為是一個(gè)羥基類(lèi)固醇脫氫酶。它表達(dá)在正常組織，但是，在阿爾茲海默斯病神經(jīng)損傷時(shí)過(guò)渡表達(dá)，在培養(yǎng)細(xì)胞中當(dāng)Aβ的毒性作用增加是過(guò)度表達(dá)。

內(nèi)質(zhì)網(wǎng)Aβ相關(guān)結(jié)合蛋白抗體產(chǎn)品應(yīng)用：ELISA=1:500-1000 IHC-P=1:100-500 IHC-F=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.

研究領(lǐng)域：**學(xué)  神經(jīng)生物學(xué)  線粒體  

儲(chǔ)存條件： Store at -20 °C for one year. Avoid repeated freeze/thaw cycles.

來(lái)源： Mouse

外觀： Lyophilized or Liquid

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