Puccio H - Search Results

1

The in vivo mitochondrial two-step maturation of human frataxin.

Schmucker S, Argentini M, Carelle-Calmels N, Martelli A, Puccio H. Schmucker S, et al. Among authors: puccio h. Hum Mol Genet. 2008 Nov 15;17(22):3521-31. doi: 10.1093/hmg/ddn244. Epub 2008 Aug 25. Hum Mol Genet. 2008. PMID: 18725397

2

Recent advances in the molecular pathogenesis of Friedreich ataxia.

Puccio H, Koenig M. Puccio H, et al. Hum Mol Genet. 2000 Apr 12;9(6):887-92. doi: 10.1093/hmg/9.6.887. Hum Mol Genet. 2000. PMID: 10767311 Review.

3

Inactivation of the Friedreich ataxia mouse gene leads to early embryonic lethality without iron accumulation.

Cossée M, Puccio H, Gansmuller A, Koutnikova H, Dierich A, LeMeur M, Fischbeck K, Dollé P, Koenig M. Cossée M, et al. Among authors: puccio h. Hum Mol Genet. 2000 May 1;9(8):1219-26. doi: 10.1093/hmg/9.8.1219. Hum Mol Genet. 2000. PMID: 10767347

4

Mouse models for Friedreich ataxia exhibit cardiomyopathy, sensory nerve defect and Fe-S enzyme deficiency followed by intramitochondrial iron deposits.

Puccio H, Simon D, Cossée M, Criqui-Filipe P, Tiziano F, Melki J, Hindelang C, Matyas R, Rustin P, Koenig M. Puccio H, et al. Nat Genet. 2001 Feb;27(2):181-6. doi: 10.1038/84818. Nat Genet. 2001. PMID: 11175786

5

Disabled early recruitment of antioxidant defenses in Friedreich's ataxia.

Chantrel-Groussard K, Geromel V, Puccio H, Koenig M, Munnich A, Rötig A, Rustin P. Chantrel-Groussard K, et al. Among authors: puccio h. Hum Mol Genet. 2001 Sep 15;10(19):2061-7. doi: 10.1093/hmg/10.19.2061. Hum Mol Genet. 2001. PMID: 11590123

6

Friedreich ataxia: a paradigm for mitochondrial diseases.

Puccio H, Koenig M. Puccio H, et al. Curr Opin Genet Dev. 2002 Jun;12(3):272-7. doi: 10.1016/s0959-437x(02)00298-8. Curr Opin Genet Dev. 2002. PMID: 12076669 Review.

7

2003 International Friedreich's Ataxia Research Conference, 14-16 February 2003, Bethesda, MD, USA.

Seznec H, Wilson RB, Puccio H. Seznec H, et al. Among authors: puccio h. Neuromuscul Disord. 2004 Jan;14(1):70-82. doi: 10.1016/j.nmd.2003.10.002. Neuromuscul Disord. 2004. PMID: 14659415 No abstract available.

8

Friedreich ataxia mouse models with progressive cerebellar and sensory ataxia reveal autophagic neurodegeneration in dorsal root ganglia.

Simon D, Seznec H, Gansmuller A, Carelle N, Weber P, Metzger D, Rustin P, Koenig M, Puccio H. Simon D, et al. Among authors: puccio h. J Neurosci. 2004 Feb 25;24(8):1987-95. doi: 10.1523/JNEUROSCI.4549-03.2004. J Neurosci. 2004. PMID: 14985441 Free PMC article.

9

Idebenone delays the onset of cardiac functional alteration without correction of Fe-S enzymes deficit in a mouse model for Friedreich ataxia.

Seznec H, Simon D, Monassier L, Criqui-Filipe P, Gansmuller A, Rustin P, Koenig M, Puccio H. Seznec H, et al. Among authors: puccio h. Hum Mol Genet. 2004 May 15;13(10):1017-24. doi: 10.1093/hmg/ddh114. Epub 2004 Mar 17. Hum Mol Genet. 2004. PMID: 15028670

10

Friedreich ataxia: the oxidative stress paradox.

Seznec H, Simon D, Bouton C, Reutenauer L, Hertzog A, Golik P, Procaccio V, Patel M, Drapier JC, Koenig M, Puccio H. Seznec H, et al. Among authors: puccio h. Hum Mol Genet. 2005 Feb 15;14(4):463-74. doi: 10.1093/hmg/ddi042. Epub 2004 Dec 22. Hum Mol Genet. 2005. PMID: 15615771

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