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CHCHD4 (基因名), Mitochondrial intermembrane space import and assembly protein 40 (蛋白名), MIA40_HUMAN.
产品名称:

Human CHCHD4/ Mitochondrial intermembrane space import and assembly protein 40 Recombinant Protein

货号:
-
商标:
EIAab®
监管等级:
别名:

Coiled-coil-helix-coiled-coil-helix domain-containing protein 4, MIA40

序列号:
Q8N4Q1
来源:
E.coli
种属:
Human
标签:
His
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:
-
Human CHCHD4 Protein
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Human CHCHD4 Protein
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产品说明书
说明书: 下载说明书
MSDS: MSDS


基因位点
Cytogenetic band: 3p25.1 by HGNC 3p25.1 by Entrez Gene 3p25.1 by Ensembl
CHCHD4 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:
Monomer (PubMed:19182799). Can form homooligomers (PubMed:16185709). Interacts with GFER and forms transient disulfide bonds with GFER (PubMed:23186364). Interacts with MICU1 (PubMed:26387864).


功能:
Functions as chaperone and catalyzes the formation of disulfide bonds in substrate proteins, such as COX17 or MICU1 (PubMed:16185709, PubMed:26387864, PubMed:19182799, PubMed:21059946, PubMed:23186364). Required for the import and folding of small cysteine-containing proteins (small Tim) in the mitochondrial intermembrane space (IMS) (PubMed:16185709, PubMed:19182799, PubMed:21059946). Precursor proteins to be imported into the IMS are translocated in their reduced form into the mitochondria (PubMed:16185709, PubMed:19182799, PubMed:21059946). The oxidized form of CHCHD4/MIA40 forms a transient intermolecular disulfide bridge with the reduced precursor protein, resulting in oxidation of the precursor protein that now contains an intramolecular disulfide bond and is able to undergo folding in the IMS (PubMed:16185709, PubMed:19182799, PubMed:21059946). Reduced CHCHD4/MIA40 is then reoxidized by GFER/ERV1 via a disulfide relay system (PubMed:23186364). Mediates formation of disulfide bond in MICU1 in the IMS, promoting formation of the MICU1-MICU2 heterodimer that regulates mitochondrial calcium uptake (PubMed:26387864).


亚细胞位置:
Mitochondrion intermembrane space


该产品尚未在任何出版物中被引用。

[1].
"The Ca(2+)-Dependent Release of the Mia40-Induced MICU1-MICU2 Dimer from MCU Regulates Mitochondrial Ca(2+) Uptake."

[2].
"Protein import and oxidative folding in the mitochondrial intermembrane space of intact mammalian cells."

[3].
"Disulfide bond formation: sulfhydryl oxidase ALR controls mitochondrial biogenesis of human MIA40."

[4].
"Human CHCHD4 mitochondrial proteins regulate cellular oxygen consumption rate and metabolism and provide a critical role in hypoxia signaling and tumor progression."

[5].
"Genetic variants in nuclear-encoded mitochondrial genes influence AIDS progression."

[6].
"Molecular chaperone function of Mia40 triggers consecutive induced folding steps of the substrate in mitochondrial protein import."

[7].
"MIA40 is an oxidoreductase that catalyzes oxidative protein folding in mitochondria."

[8].
"Functional and mutational characterization of human MIA40 acting during import into the mitochondrial intermembrane space."

[9].
"Complete sequencing and characterization of 21,243 full-length human cDNAs."

[10].
"The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC)."
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