Mössbauer studies of frataxin role in iron-sulfur cluster assembly and dysfunction-related disease

被引：0

作者：

Ricardo Garcia-Serres

Martin Clémancey

Jean-Louis Oddou

Annalisa Pastore

Emmanuel Lesuisse

Jean-Marc Latour

机构：

[1] Université Joseph Fourier,

[2] CNRS,undefined

[3] UMR5249,undefined

[4] Medical Research Council National Institute for Medical Research,undefined

[5] Laboratoire Mitochondries,undefined

[6] Métaux et Stress oxydant,undefined

[7] Institut Jacques Monod,undefined

[8] CNRS-Université Paris,undefined

[9] CEA,undefined

[10] iRTSV,undefined

[11] LCBM,undefined

来源：

Hyperfine Interactions | 2012年 / 206卷

关键词：

Mössbauer spectroscopy; Frataxin; Iron sulfur cluster; Iron accumulation;

D O I：

暂无

中图分类号：

学科分类号：

摘要：

Friedreich ataxia is a disease that is associated with defects in the gene coding for a small protein frataxin. Several different roles have been proposed for the protein, including iron chaperoning and iron storage. Mössbauer spectroscopy was used to probe these hypotheses. Iron accumulation in mutant mitochondria unable to assemble iron sulfur clusters proved to be insensitive to overexpression of frataxin, ruling out its potential involvement as an iron storage protein similar to ferritin. Rather, it was found that frataxin negatively regulates iron sulfur cluster assembly.

引用

页码：13 / 18

页数：5

共 42 条

[21] Respiratory chain supercomplexes associate with the cysteine desulfurase complex of the iron-sulfur cluster assembly machinery
Boettinger, Lena
Martensson, Christoph U.
Song, Jiyao
Zufall, Nicole
Wiedemann, Nils
Becker, Thomas
MOLECULAR BIOLOGY OF THE CELL, 2018, 29 (07) : 776 - 785
[22] Comparative structural modeling of a monothiol GRX from chickpea: Insight in iron-sulfur cluster assembly
Yadav, Saurabh
Kushwaha, Hemant Ritturaj
Kumar, Kamal
Verma, Praveen Kumar
INTERNATIONAL JOURNAL OF BIOLOGICAL MACROMOLECULES, 2012, 51 (03) : 266 - 273
[23] Expression, purification and characterization of an iron-sulfur cluster assembly protein, IscU, from Acidithiobacillus ferrooxidans
Jia Zeng
Wenjie Zhao
Yuandong Liu
Lexian Xia
Jianshe Liu
Guanzhou Qiu
Biotechnology Letters, 2007, 29 : 1965 - 1972
[24] Mitochondrial functional interactions between frataxin and Isu1p, the iron-sulfur cluster scaffold protein, in Saccharomyces cerevisiae
Ramazzotti, A
Vanmansart, V
Foury, F
FEBS LETTERS, 2004, 557 (1-3) : 215 - 220
[25] Expression, Purification, and Characterization of Iron-Sulfur Cluster Assembly Regulator IscR from Acidithiobacillus ferrooxidans
Zeng, Jia
Zhang, Ke
Liu, Jianshe
Qiu, Guanzhou
JOURNAL OF MICROBIOLOGY AND BIOTECHNOLOGY, 2008, 18 (10) : 1672 - 1677
[26] Expression, purification and characterization of an iron-sulfur cluster assembly protein, IscU, from Acidithiobacillus ferrooxidans
Zeng, Jia
Zhao, Wenjie
Liu, Yuandong
Xia, Lexian
Liu, Jianshe
Qiu, Guanzhou
BIOTECHNOLOGY LETTERS, 2007, 29 (12) : 1965 - 1972
[27] Maturation and Assembly of Iron-Sulfur Cluster-Containing Subunits in the Mitochondrial Complex I From Plants
Lopez-Lopez, Alicia
Keech, Olivier
Rouhier, Nicolas
FRONTIERS IN PLANT SCIENCE, 2022, 13
[28] Two-stage binding of mitochondrial ferredoxin-2 to the core iron-sulfur cluster assembly complex
Steinhilper, Ralf
Boss, Linda
Freibert, Sven-A.
Schulz, Vinzent
Krapoth, Nils
Kaltwasser, Susann
Lill, Roland
Murphy, Bonnie J.
NATURE COMMUNICATIONS, 2024, 15 (01)
[29] Mitochondrial type iron-sulfur cluster assembly in the amitochondriate eukaryotes Trichomonas vaginalis and Giardia intestinalis, as indicated by the phylogeny of IscS
Tachezy, J
Sánchez, LB
Müller, M
MOLECULAR BIOLOGY AND EVOLUTION, 2001, 18 (10) : 1919 - 1928
[30] Crystal structure of Escherichia coli SufC, an ABC-type ATPase component of the SUF iron-sulfur cluster assembly machinery
Kitaoka, S
Wada, K
Hasegawa, Y
Minami, Y
Fukuyama, K
Takahashi, Y
FEBS LETTERS, 2006, 580 (01) : 137 - 143

← 1 2 3 4 5 →