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Paediatric‐onset neuronal ceroid lipofuscinosis: first symptoms and presentation at diagnosis

Developmental medicine and child neurology, 2020-04, Vol.62 (4), p.528-530

2020

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De bons sentiments

Critique (Paris), 2022, Vol.n° 899 (4), p.290-298

Puech, Jean-Benoît

2022

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Barth syndrome: Cellular compensation of mitochondrial dysfunction and apoptosis inhibition due to changes in cardiolipin remodeling linked to tafazzin (TAZ) gene mutation

Biochimica et biophysica acta, 2013-08, Vol.1832 (8), p.1194-1206

2013

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Influence of Geographical Origin and Botanical Species on the Content of Extractives in American, French, and East European Oak Woods

Journal of agricultural and food chemistry, 2006-10, Vol.54 (21), p.8115-8126

2006

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Inhibition of Lysosome Membrane Recycling Causes Accumulation of Gangliosides that Contribute to Neurodegeneration

Cell reports (Cambridge), 2018-06, Vol.23 (13), p.3813-3826

2018

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canine Arylsulfatase G (ARSG) mutation leading to a sulfatase deficiency is associated with neuronal ceroid lipofuscinosis

Proceedings of the National Academy of Sciences - PNAS, 2010-08, Vol.107 (33), p.14775-14780

2010

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Intravenous administration of scAAV9-Hexb normalizes lifespan and prevents pathology in Sandhoff disease mice

Human molecular genetics, 2018-03, Vol.27 (6), p.954-968

2018

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Characterization of seven novel mutations on the HEXB gene in French Sandhoff patients

Gene, 2013-01, Vol.512 (2), p.521-526

2013

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Relation between chemical composition of oak wood used in cooperage and sensory perception of model extracts

Journal of the science of food and agriculture, 2009-03, Vol.89 (5), p.765-773

2009

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Rapid identification of HEXA mutations in Tay-Sachs patients

Biochemical and biophysical research communications, 2010-02, Vol.392 (4), p.599-602

2010

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Quel amont et quel aval pour le roman de Jordane ?

Littérature (Paris. 1971), 2021-06, Vol.N° 202 (2), p.24-39

Puech, Jean-Benoît

2021

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Jean-Benoît Puech, Vie du mensonge et vérité du roman: Propos recueillis par Jochen Mecke

Revue critique de fixxion française contemporaine, 2021-06 (22)

2021

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Figurines

Poétique, 2019-05, Vol.n° 185 (1), p.161-183

Puech, Jean-Benoît

2019

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Variation in wood volatile compounds in a mixed oak stand : strong species and spatial differentiation in whisky-lactone content

Annals of forest science., 2007-04, Vol.64 (3), p.313-320

2007

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Effect of species and ecological conditions on ellagitannin content in oak wood from an even-aged and mixed stand of Quercus robur L. and Quercus petraea Liebl

Annals of forest science., 2006-05, Vol.63 (4), p.415-424

2006

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Identification of a Precursor of β-Methyl-γ-octalactone in the Wood of Sessile Oak (Quercus petraea (Matt.) Liebl.)

Journal of agricultural and food chemistry, 2000-09, Vol.48 (9), p.4306-4309

2000

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Cork and alcoholic beverages

Food reviews international, 2005, Vol.21 (1), p.53-68

2005

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Evolution of Castalagin and Vescalagin in Ethanol Solutions. Identification of New Derivatives

Journal of agricultural and food chemistry, 1999-05, Vol.47 (5), p.2060-2066

1999

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Adenovirus-Mediated Transfer of the Acid α-Glucosidase Gene into Fibroblasts, Myoblasts and Myotubes from Patients with Glycogen Storage Disease Type II Leads to High Level Expression of Enzyme and Corrects Glycogen Accumulation

Human molecular genetics, 1998-10, Vol.7 (11), p.1695-1702

1998

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Exhaustive Screening of the Acid β-Glucosidase Gene, by Fluorescence-Assisted Mismatch Analysis Using Universal Primers: Mutation Profile and Genotype/Phenotype Correlations in Gaucher Disease

American journal of human genetics, 1998-08, Vol.63 (2), p.415-427

1998

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Mise au point d’un outil d’analyse quantitative de la perte neuronale dopaminergique du striatum en médecine nucléaire

Revue neurologique, 2019-04, Vol.175, p.S106-S107

2019

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50 activités pour apprivoiser Internet à l'école et au collège

1998

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Comparison of direct and indirect methods of measuring the precursors of β-methyl-γ-octalactone and their application to the analysis of Sessile oak wood [ Quercus petraea (Matt.) Liebl.]

Journal of Chromatography A, 2001-01, Vol.905 (1), p.183-191

2001

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Quality Assessment of Oenological Tannins Utilising Global Selectivity Chemical Sensors Array (“Electronic Tongue”)

South African Journal of Enology & Viticulture, 2016-12, Vol.28 (2)

2016

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Localization of the ellagitannins in the tissues of Quercus robur and Quercus petraea woods

Phytochemistry (Oxford), 1994-11, Vol.37 (5), p.1245-1249

1994

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Paediatric‐onset neuronal ceroid lipofuscinosis: first symptoms and presentation at diagnosis

De bons sentiments

Barth syndrome: Cellular compensation of mitochondrial dysfunction and apoptosis inhibition due to changes in cardiolipin remodeling linked to tafazzin (TAZ) gene mutation

Influence of Geographical Origin and Botanical Species on the Content of Extractives in American, French, and East European Oak Woods

Inhibition of Lysosome Membrane Recycling Causes Accumulation of Gangliosides that Contribute to Neurodegeneration

canine Arylsulfatase G (ARSG) mutation leading to a sulfatase deficiency is associated with neuronal ceroid lipofuscinosis

Intravenous administration of scAAV9-Hexb normalizes lifespan and prevents pathology in Sandhoff disease mice

Characterization of seven novel mutations on the HEXB gene in French Sandhoff patients

Relation between chemical composition of oak wood used in cooperage and sensory perception of model extracts

Rapid identification of HEXA mutations in Tay-Sachs patients

Quel amont et quel aval pour le roman de Jordane ?

Jean-Benoît Puech, Vie du mensonge et vérité du roman: Propos recueillis par Jochen Mecke

Figurines

Variation in wood volatile compounds in a mixed oak stand : strong species and spatial differentiation in whisky-lactone content

Effect of species and ecological conditions on ellagitannin content in oak wood from an even-aged and mixed stand of Quercus robur L. and Quercus petraea Liebl

Identification of a Precursor of β-Methyl-γ-octalactone in the Wood of Sessile Oak (Quercus petraea (Matt.) Liebl.)

Cork and alcoholic beverages

Evolution of Castalagin and Vescalagin in Ethanol Solutions. Identification of New Derivatives

Adenovirus-Mediated Transfer of the Acid α-Glucosidase Gene into Fibroblasts, Myoblasts and Myotubes from Patients with Glycogen Storage Disease Type II Leads to High Level Expression of Enzyme and Corrects Glycogen Accumulation

Exhaustive Screening of the Acid β-Glucosidase Gene, by Fluorescence-Assisted Mismatch Analysis Using Universal Primers: Mutation Profile and Genotype/Phenotype Correlations in Gaucher Disease

Mise au point d’un outil d’analyse quantitative de la perte neuronale dopaminergique du striatum en médecine nucléaire

50 activités pour apprivoiser Internet à l'école et au collège

Comparison of direct and indirect methods of measuring the precursors of β-methyl-γ-octalactone and their application to the analysis of Sessile oak wood [ Quercus petraea (Matt.) Liebl.]

Quality Assessment of Oenological Tannins Utilising Global Selectivity Chemical Sensors Array (“Electronic Tongue”)

Localization of the ellagitannins in the tissues of Quercus robur and Quercus petraea woods

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