Výsledky vyhledávání - "Marion Paturneau-Jouas"

Assessing gene and cell therapies applied in striated skeletal and cardiac muscle: Is there a role for nuclear magnetic resonance?

Autor: Marion Paturneau-Jouas, Anne Leroy-Willig, Yves Fromes, Pierre G. Carlier

Publikováno v: Neuromuscular Disorders. 13:397-407

Gene and cell therapies convey high hopes for treatment of skeletal and heart muscle diseases. In the experimental protocols under development as well as in the first clinical trials, longitudinal control by an atraumatic procedure is needed. Nuclear

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::8cd5636622ded5cf67062d4809c6927b
https://doi.org/10.1016/s0960-8966(03)00035-x

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Electrotransfer of naked DNA in the skeletal muscles of animal models of muscular dystrophies

Autor: Delaere P, Daniel Scherman, Ketty Schwartz, Marion Paturneau-Jouas, Pierre Chapdelaine, Jacques P. Tremblay, Marc Fiszman, N Boissel, P F Kennel, Jean-Thomas Vilquin

Publikováno v: Gene Therapy. 8:1097-1107

The electrotransfer of naked DNA has recently been adapted to the transduction of skeletal muscle fibers. We investigated the short- and long-term efficacy of this methodology in wild-type animals and in mouse models of congenital muscular dystrophy

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::3ff18e76a1b5468c35eff0d0414a8b0e
https://doi.org/10.1038/sj.gt.3301484

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Inhibition of Fatty Acid β-Oxidation in Rat Brain Cultured Astrocytes Exposed to the Neurotoxin 3-Nitropropionic Acid

Autor: Marion Paturneau-Jouas, Dhoinine Soifiyoudine, Ali Esfandiari

Publikováno v: Developmental Neuroscience. 19:312-320

We describe the effects of the neurotoxin 3-nitropropionic acid (3-NPA) on fatty acid oxidation in neonatal rat brain astrocytes in primary culture, using a sensitive assay for beta-oxidation which depends on the release of 3H2O from [9,10(n)-3H]palm

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c8830f4274462218ab4c7be354d1e5a0
https://doi.org/10.1159/000111227

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Deletions of mitochondrial DNA in Kearns-Sayre syndrome and ocular myopathies: Genetic, biochemical and morphological studies

Autor: J.P. Leroux, Isabelle Nelson, Michel Fardeau, Norma B. Romero, G. Ponsot, Marion Paturneau-Jouas, Bruno Eymard, Dominique François, Cécile Marsac, Denis Duboc, Patrick Lestienne, Françoise Degoul, Chaussain M

Publikováno v: Journal of the Neurological Sciences. 101:168-177

Genetic, biochemical and morphological investigations were conducted on skeletal muscle mitochondria from 6 cases of ocular myopathy: 4 cases with Kearns-Sayre syndrome (KSS) and 2 with chronic progressive external ophthalmoplegia. All of these 6 cas

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https://doi.org/10.1016/0022-510x(91)90042-6

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Drug-induced readthrough of premature stop codons leads to the stabilization of laminin alpha2 chain mRNA in CMD myotubes

Autor: Ryoichi Matsuda, Masayuki Arakawa, Célia Floquet, Laure Bidou, C. Gartioux, Valérie Allamand, Masataka Shiozuka, Pascale Guicheney, Jean Pierre Rousset, Gillian Butler-Browne, Daishiro Ikeda, Vincent Mouly, Marion Paturneau-Jouas

Publikováno v: The Journal of Gene Medicine
The Journal of Gene Medicine, Wiley, 2007, epub ahead of print. ⟨10.1002/jgm.1140⟩

BACKGROUND: The most common form of congenital muscular dystrophy is caused by a deficiency in the alpha2 chain of laminin-211, a protein of the extracellular matrix. A wide variety of mutations, including 20 to 30% of nonsense mutations, have been i

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https://pubmed.ncbi.nlm.nih.gov/18074402

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Tubular aggregates are from whole sarcoplasmic reticulum origin: alterations in calcium binding protein expression in mouse skeletal muscle during aging

Autor: Martine Verdière-Sahuqué, I Marty, Daniel Hantaï, Marion Paturneau-Jouas, Frédéric Chevessier

Publikováno v: Neuromuscular disorders : NMD. 14(3)

Tubular aggregates are observed in various muscle disorders and appear as densely packed tubules believed to arise from sarcoplasmic reticulum of striated muscle. They are found both in human skeletal muscle, especially from patients suffering from '

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https://pubmed.ncbi.nlm.nih.gov/15036331

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Contents, Vol. 58, Supplement 1, 1990

Publikováno v: Neonatology. 58:I-III

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https://doi.org/10.1159/000243295

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Effects of Inhibitors of the Mitochondrial Electron Transport Chain on Fatty Acid Beta-Oxidation in Rat Brain Cultured Astrocytes

Autor: Marion Paturneau-Jouas, Ali Esfandiari

Publikováno v: Neurochemistry ISBN: 9781461374688

The high energy electrons generated by the oxidation of NADH and FADH2 are transferred through the mitochondrial respiratory chain, leading to the production of ATP, the main source of energy in the cells. Dysfunction of the respiratory chain occurs

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https://doi.org/10.1007/978-1-4615-5405-9_124

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Adrenoleukodystrophy in France: an epidemiological study

Autor: Nicole Baumann, Carole Sereni, Patrick Aubourg, Josué Feingold, Marion Paturneau-Jouas

Publikováno v: Neuroepidemiology. 12(4)

An epidemiological study was conducted in France to estimate the prevalence of adrenoleukodystrophy, a severe neurologic X-linked disorder affecting boys and young men. 129 cases were collected. Analysis of all cases born between 1956 and 1986 with a

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https://pubmed.ncbi.nlm.nih.gov/8272182

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