ISCA1 is essential for mitochondrial Fe4S4 biogenesis in vivo

被引：82

作者：

Beilschmidt, Lena Kristina ^{[1
,2
,3
,4
,5
]}

de Choudens, Sandrine Ollagnier ^{[6
,7
,8
]}

Fournier, Marjorie ^{[1
,2
,3
,4
,11
]}

Sanakis, Ioannis ^{[9
]}

Hograindleur, Marc-Andre ^{[6
,7
,8
,10
]}

Clemancey, Martin ^{[7
,8
,10
]}

Blondin, Genevieve ^{[7
,8
,10
]}

Schmucker, Stephane ^{[1
,2
,3
,4
,5
]}

Eisenmann, Aurelie ^{[1
,2
,3
,4
,5
]}

Weiss, Amelie ^{[1
,2
,3
,4
]}

Koebel, Pascale ^{[1
,2
,3
,4
]}

Messaddeq, Nadia ^{[1
,2
,3
,4
]}

Puccio, Helene ^{[1
,2
,3
,4
,5
]}

Martelli, Alain ^{[1
,2
,3
,4
,5
,12
]}

机构：

[1] IGBMC, Translat Med & Neurogenet Dept, F-67404 Illkirch Graffenstaden, France

[2] INSERM, U596, F-67404 Illkirch Graffenstaden, France

[3] CNRS, UMR7104, F-67404 Illkirch Graffenstaden, France

[4] Univ Strasbourg, F-67000 Strasbourg, France

[5] Coll France, Chaire Genet Humaine, F-67404 Illkirch Graffenstaden, France

[6] CEA DRF BIG CBM BioCat, F-38054 Grenoble, France

[7] CNRS, UMR 5249, LCBM, F-38054 Grenoble, France

[8] Univ Grenoble Alpes, LCBM, F-38054 Grenoble, France

[9] NCSR, Inst Mat Sci, Attiki, Greece

[10] CEA DRF BIG CBM Pmb, F-38054 Grenoble, France

[11] Univ Oxford, Sir William Dunn Sch Pathol, Oxford OX1 3RE, England

[12] Pfizer Inc, Rare Dis Res Unit, Cambridge, MA 02139 USA

来源：

NATURE COMMUNICATIONS | 2017年 / 8卷

基金：

欧洲研究理事会;

关键词：

IRON-SULFUR CLUSTER; FE-S PROTEINS; ESCHERICHIA-COLI; SACCHAROMYCES-CEREVISIAE; MOSSBAUER-SPECTROSCOPY; AZOTOBACTER-VINELANDII; HUMAN FRATAXIN; SCAFFOLD PROTEIN; BIOTIN SYNTHASE; 4FE-4S CLUSTER;

D O I：

10.1038/ncomms15124

中图分类号：

O [数理科学和化学]; P [天文学、地球科学]; Q [生物科学]; N [自然科学总论];

学科分类号：

07 ; 0710 ; 09 ;

摘要：

Mammalian A-type proteins, ISCA1 and ISCA2, are evolutionarily conserved proteins involved in iron-sulfur cluster (Fe-S) biogenesis. Recently, it was shown that ISCA1 and ISCA2 form a heterocomplex that is implicated in the maturation of mitochondrial Fe4S4 proteins. Here we report that mouse ISCA1 and ISCA2 are Fe2S2-containing proteins that combine all features of Fe-S carrier proteins. We use biochemical, spectroscopic and in vivo approaches to demonstrate that despite forming a complex, ISCA1 and ISCA2 establish discrete interactions with components of the late Fe-S machinery. Surprisingly, knockdown experiments in mouse skeletal muscle and in primary cultures of neurons suggest that ISCA1, but not ISCA2, is required for mitochondrial Fe4S4 proteins biogenesis. Collectively, our data suggest that cellular processes with different requirements for ISCA1, ISCA2 and ISCA1-ISCA2 complex seem to exist.

引用

页数：12

共 59 条

[1] ISCA2 mutation causes infantile neurodegenerative mitochondrial disorder [J].

Al-Hassnan, Zuhair N. ;

Al-Dosary, Mazhor ;

Alfadhel, Majid ;

Faqeih, Eissa A. ;

Alsagob, Maysoon ;

Kenana, Rosan ;

Almass, Rawan ;

Al-Harazi, Olfat S. ;

Al-Hindi, Hindi ;

Malibari, Omhani I. ;

Almutari, Faten B. ;

Tulbah, Sahar ;

Alhadeq, Faten ;

Al-Sheddi, Tarfa ;

Alamro, Rana ;

AlAsmari, Ali ;

Almuntashri, Makki ;

Alshaalan, Hesham ;

Al-Mohanna, Futwan A. ;

Colak, Dilek ;

Kaya, Namik .

JOURNAL OF MEDICAL GENETICS, 2015, 52 (03) :186-194

[2] NfuA, a new factor required for maturing Fe/S proteins in Escherichia coli under oxidative stress and iron starvation conditions [J].

Angelini, Sandra ;

Gerez, Catherine ;

Ollagnier-de Choudens, Sandrine ;

Sanakis, Yiannis ;

Fontecave, Marc ;

Barras, Frederic ;

Py, Beatrice .

JOURNAL OF BIOLOGICAL CHEMISTRY, 2008, 283 (20) :14084-14091

[3] [2Fe-2S] cluster transfer in iron-sulfur protein biogenesis [J].

Banci, Lucia ;

Brancaccio, Diego ;

Ciofi-Baffoni, Simone ;

Del Conte, Rebecca ;

Gadepalli, Ravisekhar ;

Mikolajczyk, Maciej ;

Neri, Sara ;

Piccioli, Mario ;

Winkelmann, Julia .

PROCEEDINGS OF THE NATIONAL ACADEMY OF SCIENCES OF THE UNITED STATES OF AMERICA, 2014, 111 (17) :6203-6208

[4] A proposed role for the Azotobacter vinelandii NfuA protein as an intermediate iron-sulfur cluster carrier [J].

Bandyopadhyay, Sibali ;

Naik, Sunil G. ;

O'Carroll, Ina P. ;

Huynh, Boi-Hanh ;

Dean, Dennis R. ;

Johnson, Michael K. ;

Dos Santos, Patricia C. .

JOURNAL OF BIOLOGICAL CHEMISTRY, 2008, 283 (20) :14092-14099

[5] Mammalian Fe-S cluster biogenesis and its implication in disease [J].

Beilschmidt, Lena K. ;

Puccio, Helene M. .

BIOCHIMIE, 2014, 100 :48-60

[6] Iron-sulfur clusters of biotin synthase in vivo:: A Mossbauer Study [J].

Benda, R ;

Bui, BTS ;

Schünemann, V ;

Florentin, D ;

Marquet, A ;

Trautwein, AX .

BIOCHEMISTRY, 2002, 41 (50) :15000-15006

[7] Mutation of the iron-sulfur cluster assembly gene IBA57 causes severe myopathy and encephalopathy [J].

Bolar, Nikhita Ajit ;

Vanlander, Arnaud Vincent ;

Wilbrecht, Claudia ;

Van der Aa, Nathalie ;

Smet, Joel ;

De Paepe, Boel ;

Vandeweyer, Geert ;

Kooy, Frank ;

Eyskens, Francois ;

De Latter, Elien ;

Delanghe, Gwenda ;

Govaert, Paul ;

Leroy, Jules Gerard ;

Loeys, Bart ;

Lill, Roland ;

Van Laer, Lut ;

Van Coster, Rudy .

HUMAN MOLECULAR GENETICS, 2013, 22 (13) :2590-2602

[8] Formation of [4Fe-4S] Clusters in the Mitochondrial Iron-Sulfur Cluster Assembly Machinery [J].

Brancaccio, Diego ;

Gallo, Angelo ;

Mikolajczyk, Maciej ;

Zovo, Kairit ;

Palumaa, Peep ;

Novellino, Ettore ;

Piccioli, Mario ;

Ciofi-Baffoni, Simone ;

Banci, Lucia .

JOURNAL OF THE AMERICAN CHEMICAL SOCIETY, 2014, 136 (46) :16240-16250

[9] Human Frataxin Activates Fe-S Cluster Biosynthesis by Facilitating Sulfur Transfer Chemistry [J].

Bridwell-Rabb, Jennifer ;

Fox, Nicholas G. ;

Tsai, Chi-Lin ;

Winn, Andrew M. ;

Barondeau, David P. .

BIOCHEMISTRY, 2014, 53 (30) :4904-4913

[10] Mutations in Iron-Sulfur Cluster Scaffold Genes NFU1 and BOLA3 Cause a Fatal Deficiency of Multiple Respiratory Chain and 2-Oxoacid Dehydrogenase Enzymes [J].

Cameron, Jessie M. ;

Janer, Alexandre ;

Levandovskiy, Valeriy ;

MacKay, Nevena ;

Rouault, Tracey A. ;

Tong, Wing-Hang ;

Ogilvie, Isla ;

Shoubridge, Eric A. ;

Robinson, Brian H. .

AMERICAN JOURNAL OF HUMAN GENETICS, 2011, 89 (04) :486-495

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