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首页  >  重组蛋白  >  Human HSD17B10 Recombinant Protein
HSD17B10 (基因名), 3-hydroxyacyl-CoA dehydrogenase type-2 (蛋白名), hcd2_human.
产品名称:

Human HSD17B10/ 3-hydroxyacyl-CoA dehydrogenase type-2 Recombinant Protein
3-羟酰辅酶A脱氢酶2

货号:

R5607h

商标:
EIAab®
监管等级:
别名:

17-beta-hydroxysteroid dehydrogenase 10, 2-methyl-3-hydroxybutyryl-CoA dehydrogenase, 3-hydroxy-2-methylbutyryl-CoA dehydrogenase, 3-hydroxyacyl-CoA dehydrogenase type II, Endoplasmic reticulum-associated amyloid beta-peptide-binding protein, Mitochondrial ribonuclease P protein 2, Short chain dehydrogenase/reductase family 5C member 1, Short-chain type dehydrogenase/reductase XH98G2, Type II HADH, 17-beta-HSD 10, MHBD, Mitochondrial RNase P protein 2, ERAB, HADH2, MRPP2, SCHAD, SDR5C1, XH98G2

序列号:
Q99714
来源:
E.coli
种属:
Human
标签:
His
序列:
2-261aa
预估分子量:
28.6 kDa (monomer)
纯度:
>90% by SDS-PAGE
浓度:
Reconstitution Dependent
形态:
Liquid
内毒素水平:
Please contact protein@eiaab.com The technician for more information.
应用:
存储缓冲液:
50mM NaH2PO4, 500mM NaCl Buffer with 500mM Imidazole, 10%glycerol(PH8.0)
存储:
Store at -20°C. (Avoid repeated freezing and thawing.)
研究领域:
Neurosciences
Human HSD17B10 Protein
规格 & 价格: cart
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Human HSD17B10 Protein
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产品说明书
说明书: 下载说明书
MSDS: MSDS
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R&D 技术数据
更多信息,请参阅手册,或联系我们的技术支持: tech@eiaab.com.
基因位点
Cytogenetic band: Xp11.22 by HGNC Xp11.22 by Entrez Gene Xp11.22 by Ensembl
HSD17B10 Gene in genomic location: bands according to Ensembl, locations according to GeneLoc (and/or Entrez Gene and/or Ensembl if different)
基因位点
通用注释


亚单元:
Homotetramer (PubMed:15342248, PubMed:20077426, PubMed:25925575). Component of mitochondrial ribonuclease P, a complex composed of TRMT10C/MRPP1, HSD17B10/MRPP2 and PRORP/MRPP3 (PubMed:18984158, PubMed:25925575, PubMed:26950678, PubMed:28888424). Interacts with TRMT10C/MRPP1; forming the MRPP1-MRPP2 subcomplex of the mitochondrial ribonuclease P complex (PubMed:23042678, PubMed:29040705).


功能:
In addition to mitochondrial dehydrogenase activity, moonlights as a component of mitochondrial ribonuclease P, a complex that cleaves tRNA molecules in their 5'-ends (PubMed:18984158, PubMed:24549042, PubMed:25925575, PubMed:26950678, PubMed:28888424). Together with HSD17B10/MRPP2, forms a subcomplex of the mitochondrial ribonuclease P, named MRPP1-MRPP2 subcomplex, which displays functions that are independent of the ribonuclease P activity (PubMed:23042678, PubMed:29040705). The MRPP1-MRPP2 subcomplex catalyzes the formation of N(1)-methylguanine and N(1)-methyladenine at position 9 (m1G9 and m1A9, respectively) in tRNAs; HSD17B10/MRPP2 acting as a non-catalytic subunit (PubMed:23042678, PubMed:25925575, PubMed:28888424). The MRPP1-MRPP2 subcomplex also acts as a tRNA maturation platform: following 5'-end cleavage by the mitochondrial ribonuclease P complex, the MRPP1-MRPP2 subcomplex enhances the efficiency of 3'-processing catalyzed by ELAC2, retains the tRNA product after ELAC2 processing and presents the nascent tRNA to the mitochondrial CCA tRNA nucleotidyltransferase TRNT1 enzyme (PubMed:29040705).


亚细胞位置:
Mitochondrion


该产品尚未在任何出版物中被引用。

[1].
"The reduced expression of the HADH2 protein causes X-linked mental retardation, choreoathetosis, and abnormal behavior."

[2].
"An intracellular protein that binds amyloid-beta peptide and mediates neurotoxicity in Alzheimer's disease."

[3].
"A non-enzymatic function of 17beta-hydroxysteroid dehydrogenase type 10 is required for mitochondrial integrity and cell survival."

[4].
"Mental retardation linked to mutations in the HSD17B10 gene interfering with neurosteroid and isoleucine metabolism."

[5].
"RNase P without RNA: identification and functional reconstitution of the human mitochondrial tRNA processing enzyme."

[6].
"Comparative evolutionary genomics of the HADH2 gene encoding Abeta-binding alcohol dehydrogenase/17beta-hydroxysteroid dehydrogenase type 10 (ABAD/HSD10)."

[7].
"2-Methyl-3-hydroxybutyryl-CoA dehydrogenase (MHBD) deficiency: an X-linked inborn error of isoleucine metabolism that may mimic a mitochondrial disease."

[8].
"Crystal structure of human ABAD/HSD10 with a bound inhibitor: implications for design of Alzheimer's disease therapeutics."

[9].
"ABAD directly links Abeta to mitochondrial toxicity in Alzheimer's disease."

[10].
"2-Methyl-3-hydroxybutyryl-CoA dehydrogenase deficiency is caused by mutations in the HADH2 gene."
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