Zobrazeno 1 - 10
of 15
pro vyhledávání: '"Stéphane Schmucker"'
Autor:
Lena Kristina Beilschmidt, Sandrine Ollagnier de Choudens, Marjorie Fournier, Ioannis Sanakis, Marc-André Hograindleur, Martin Clémancey, Geneviève Blondin, Stéphane Schmucker, Aurélie Eisenmann, Amélie Weiss, Pascale Koebel, Nadia Messaddeq, Hélène Puccio, Alain Martelli
Publikováno v:
Nature Communications, Vol 8, Iss 1, Pp 1-12 (2017)
The mitochondrial proteins ISCA1 and ISCA2 form a complex that is involved in the biogenesis of Fe–S clusters. Here the authors report that ISCA1 and ISCA2 interact differently with proteins of the Fe–S machinery and that under certain conditions
Externí odkaz:
https://doaj.org/article/067e79be2eb44b3496a3b8fba496a36b
Autor:
Stéphane Schmucker, Alain Martelli, Florent Colin, Adeline Page, Marie Wattenhofer-Donzé, Laurence Reutenauer, Hélène Puccio
Publikováno v:
PLoS ONE, Vol 6, Iss 1, p e16199 (2011)
BACKGROUND: Frataxin, the mitochondrial protein deficient in Friedreich ataxia, a rare autosomal recessive neurodegenerative disorder, is thought to be involved in multiple iron-dependent mitochondrial pathways. In particular, frataxin plays an impor
Externí odkaz:
https://doaj.org/article/c6a50afee2344fd683d3325c207d468d
Autor:
Nadège Calmels, Stéphane Schmucker, Marie Wattenhofer-Donzé, Alain Martelli, Nadège Vaucamps, Laurence Reutenauer, Nadia Messaddeq, Cécile Bouton, Michel Koenig, Hélène Puccio
Publikováno v:
PLoS ONE, Vol 4, Iss 7, p e6379 (2009)
BACKGROUND:Friedreich ataxia (FRDA), the most common form of recessive ataxia, is due to reduced levels of frataxin, a highly conserved mitochondrial iron-chaperone involved in iron-sulfur cluster (ISC) biogenesis. Most patients are homozygous for a
Externí odkaz:
https://doaj.org/article/e20e9a7c96684b6e855d8386ef61204e
Autor:
Carole Peyssonnaux, Stéphane Schmucker, Matthias W. Hentze, Bruno Galy, Alain Martelli, Hervé Puy, Laurence Reutenauer, Hélène Puccio, Jacques Mathieu, Zoubida Karim
Publikováno v:
Cell Metabolism. 21(2):311-322
SummaryMitochondrial iron accumulation is a hallmark of diseases associated with impaired iron-sulfur cluster (Fe-S) biogenesis, such as Friedreich ataxia linked to frataxin (FXN) deficiency. The pathophysiological relevance of the mitochondrial iron
Autor:
Amélie Weiss, Marc-André Hograindleur, Martin Clémancey, Ioannis Sanakis, Hélène Puccio, Marjorie Fournier, Sandrine Ollagnier de Choudens, Pascale Koebel, Stéphane Schmucker, Aurélie Eisenmann, Geneviève Blondin, Nadia Messaddeq, Lena Kristina Beilschmidt, Alain Martelli
Publikováno v:
Nature Communications
Nature Communications, Nature Publishing Group, 2017, 8 (15124), ⟨10.1038/ncomms15124⟩
Nature Communications, Vol 8, Iss 1, Pp 1-12 (2017)
Nature Communications, 2017, 8 (15124), ⟨10.1038/ncomms15124⟩
Nature Communications, Nature Publishing Group, 2017, 8 (15124), ⟨10.1038/ncomms15124⟩
Nature Communications, Vol 8, Iss 1, Pp 1-12 (2017)
Nature Communications, 2017, 8 (15124), ⟨10.1038/ncomms15124⟩
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 mitochon
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=doi_dedup___::64eb343246c2abc5e7d7962b1bbeadee
https://hal.archives-ouvertes.fr/hal-01535806
https://hal.archives-ouvertes.fr/hal-01535806
Autor:
Lena Kristina, Beilschmidt, Sandrine, Ollagnier de Choudens, Marjorie, Fournier, Ioannis, Sanakis, Marc-André, Hograindleur, Martin, Clémancey, Geneviève, Blondin, Stéphane, Schmucker, Aurélie, Eisenmann, Amélie, Weiss, Pascale, Koebel, Nadia, Messaddeq, Hélène, Puccio, Alain, Martelli
Publikováno v:
Nature Communications
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 mitochon
Autor:
Marie Wattenhofer-Donzé, Jean-Claude Drapier, Blanche Guillon, Bertrand Friguet, Anne-Laure Bulteau, Hélène Puccio, Stéphane Schmucker, Cécile Bouton
Publikováno v:
FEBS Journal. 276:1036-1047
Friedreich ataxia (FRDA) is a rare hereditary neurodegenerative disease characterized by progressive ataxia and cardiomyopathy. The cause of the disease is a defect in mitochondrial frataxin, an iron chaperone involved in the maturation of Fe-S clust
Autor:
Hélène Puccio, Stéphane Schmucker
Publikováno v:
ChemInform. 41
Friedreich’s ataxia (FRDA) is a neurodegenerative disease caused by reduced expression of the mitochondrial protein frataxin. The physiopathological consequences of frataxin deficiency are a severe disruption of iron– sulfur cluster biosynthesis,
Autor:
Hélène Puccio, Stéphane Schmucker
Publikováno v:
Human molecular genetics. 19(R1)
Friedreich’s ataxia (FRDA) is a neurodegenerative disease caused by reduced expression of the mitochondrial protein frataxin. The physiopathological consequences of frataxin deficiency are a severe disruption of iron– sulfur cluster biosynthesis,
Autor:
Blanche, Guillon, Anne-Laure, Bulteau, Marie, Wattenhofer-Donzé, Stéphane, Schmucker, Bertrand, Friguet, Hélène, Puccio, Jean-Claude, Drapier, Cécile, Bouton
Publikováno v:
FEBS Journal
FEBS Journal, Wiley, 2009, 276 (4), pp.1036-47. ⟨10.1111/j.1742-4658.2008.06847.x⟩
FEBS Journal, Wiley, 2009, 276 (4), pp.1036-47. ⟨10.1111/j.1742-4658.2008.06847.x⟩
International audience; Friedreich ataxia (FRDA) is a rare hereditary neurodegenerative disease characterized by progressive ataxia and cardiomyopathy. The cause of the disease is a defect in mitochondrial frataxin, an iron chaperone involved in the
Externí odkaz:
https://explore.openaire.eu/search/publication?articleId=pmid_dedup__::0a0d270456a0179a84ad09489d1148fa
https://hal.archives-ouvertes.fr/hal-00365808
https://hal.archives-ouvertes.fr/hal-00365808