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F. Demaugre · SciRah

Researcher profile · Semantic Scholar

F. Demaugre

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F. Demaugre — researcher profile on SciRah

h-index: 19
Works: 46
Citations: 1,566

Most-cited papers

Carnitine palmitoyltransferase deficiencies.
J. Bonnefont, F. Demaugre, Carina Prip-Buus, J. Saudubray +3 more
1999Molecular Genetics and Metabolism232 citations
Semantic Scholar
Hepatitis C virus core protein binds to apolipoprotein AII and its secretion is modulated by fibrates
A. Sabile, G. Perlemuter, Fulvia Bono, K. Kohara +6 more
1999Hepatology192 citations Open access
Semantic Scholar
Hepatic and Muscular Presentations of Carnitine Palmitoyl Transferase Deficiency: Two Distinct Entities
F. Demaugre, J. Bonnefont, G. Mitchell, Nguyen-hoang Nam +4 more
1988Pediatric Research128 citations
Semantic Scholar
Carnitine-acylcarnitine translocase deficiency with severe hypoglycemia and auriculo ventricular block. Translocase assay in permeabilized fibroblasts.
S. Pande, M. Brivet, A. Slama, F. Demaugre +2 more
1993Journal of Clinical Investigation115 citations Open access
Semantic Scholar
Correlation between genotype, metabolic data, and clinical presentation in carnitine palmitoyltransferase 2 (CPT2) deficiency
L. Thuillier, H. Rostane, V. Droin, F. Demaugre +6 more
2003Human Mutation107 citations
Semantic Scholar
Molecular analysis of carnitine palmitoyltransferase II deficiency with hepatocardiomuscular expression.
J. Bonnefont, Franco Taroni, P. Cavadini, C. Cepanec +4 more
1996American Journal of Human Genetics91 citations
Semantic Scholar
Global proteomic approach unmasks involvement of keratins 8 and 18 in the delivery of cystic fibrosis transmembrane conductance regulator (CFTR)/ΔF508‐CFTR to the plasma membrane
N. Davezac, D. Tondelier, J. Lipecka, P. Fanen +6 more
2004Proteomics63 citations
Semantic Scholar
Brief report: renal tubular acidosis in carnitine palmitoyltransferase type 1 deficiency.
Zipora C. Falik Borenstein, Stanley C. Jordan, J. Saudubray, M. Brivet +3 more
1992New England Journal of Medicine59 citations Open access
Semantic Scholar
Defects in activation and transport of fatty acids
M. Brivet, A. Boutron, A. Slama, C. Costa +5 more
1999Journal of Inherited Metabolic Disease54 citations
Semantic Scholar
Identification of Cellular Targets in Human Intrahepatic Cholangiocarcinoma Using Laser Microdissection and Accurate Mass and Time Tag Proteomics*
A. Dos Santos, M. Court, V. Thiers, Sokhavuth Sar +6 more
2010Molecular & Cellular Proteomics52 citations Open access
Semantic Scholar
Immunoquantitative Analysis of Human Carnitine Palmitoyltransferase I and II Defects
F. Demaugre, J. Bonnefont, C. Cepanec, J. Scholte +2 more
1990Pediatric Research52 citations
Semantic Scholar
Organization of the human liver carnitine palmitoyltransferase 1 gene (CPT1A) and identification of novel mutations in hypoketotic hypoglycaemia
S. Gobin, J. Bonnefont, C. Prip-Buus, C. Mugnier +13 more
2002Human Genetics51 citations
Semantic Scholar
Dichloroacetate as treatment for congenital lactic acidosis.
F. Coudé, J. Saudubray, F. Demaugre, C. Marsac +2 more
1978New England Journal of Medicine41 citations
Semantic Scholar
Genotype/phenotype correlation in carnitine palmitoyl transferase II deficiency: lessons from a compound heterozygous patient.
L. Thuillier, C. Sevin, F. Demaugre, M. Brivet +7 more
2000Neuromuscular Disorders31 citations
Semantic Scholar
Identification of four novel mutations in patients with carnitine palmitoyltransferase II (CPT II) deficiency.
Bing-Zhi Yang, Jia-Huan Ding, T. Dewese, D. Roe +7 more
1998Molecular Genetics and Metabolism30 citations
Semantic Scholar
Deficiency of Carnitine Palmitoyltransferase I
J. Bonnefont, Richard D. Haas, Jon Wolff, Le Phuc Thuy +5 more
1989Journal of Child Neurology28 citations
Semantic Scholar
Elevated plasma carnitine in the hepatic form of carnitine palmitoyltransferase-1 deficiency
C. Stanley, F. Sunaryo, D. Hale, J. Bonnefont +2 more
1992Journal of Inherited Metabolic Disease27 citations
Semantic Scholar
Approach to the patient with a fatty acid oxidation disorder.
J. Saudubray, Grant A. Mitchell, J. Bonnefont, G. Schwartz +6 more
1992Progress in clinical and biological research26 citations
Semantic Scholar
The effects of pyruvate concentration, dichloroacetate and alpha-cyano-4-hydroxycinnamate on gluconeogenesis, ketogenesis and [3-hydroxybutyrate]/[3-oxobutyrate] ratios in isolated rat hepatocytes.
F. Demaugre, Jean-Paul Leroux, P. Cartier
1978Biochemical Journal26 citations Open access
Semantic Scholar
Evaluation of isolated human hepatocytes.
D. Houssin, M. Capron, Chantal Celier, T. Cresteil +2 more
1983Life Science19 citations
Semantic Scholar
HIP/PAP, a C-type lectin overexpressed in hepatocellular carcinoma, binds the RII alpha regulatory subunit of cAMP-dependent protein kinase and alters the cAMP-dependent protein kinase signalling.
F. Demaugre, Yannick Philippe, Sokavuth Sar, B. Pileire +3 more
2004European Journal of Biochemistry17 citations
Semantic Scholar
Phytanic acid alpha-oxidation and complementation analysis of classical Refsum and peroxisomal disorders
Bwee Tien Poll-The, O. Skjeldal, O. Stokke, A. Poulos +2 more
2004Human Genetics15 citations
Semantic Scholar
[Familial diphosphoglycerate mutase deficiency: hematological and biochemical study].
P. Cartier, D. Labie, J. Leroux, A. Najman +1 more
1972Nouvelle revue francaise d'hematologie13 citations
Semantic Scholar
Influence of oleate oxidation on pyruvate production and utilization in hepatocytes isolated from fed rats. Effect of 2-[5-(4-chlorophenyl)pentyl]oxiran-2-carboxylate.
F. Demaugre, H. Buc, Claude Cepanec, A. Moncion +1 more
1984Biochemical Journal12 citations Open access
Semantic Scholar
Complementation Analysis of Carnitine Palmitoyltransferase I and II Defects
A. Slama, M. Brivet, Audrey Boutron, A. Legrand +2 more
1996Pediatric Research11 citations
Semantic Scholar
Retrospective diagnosis of carnitine-acylcarnitine translocase deficiency by acylcarnitine analysis in the proband Guthrie card and enzymatic studies in the parents
M. Brivet, A. Slama, D. S. Millington, C. Roe +5 more
1996Journal of Inherited Metabolic Disease8 citations
Semantic Scholar
Diagnosis of carnitine acylcarnitine translocase deficiency by complementation analysis
M. Brivet, A. Slama, H. Ogier, A. Boutron +3 more
1994Journal of Inherited Metabolic Disease8 citations
Semantic Scholar
Influence of oxalate on the rate of the tricarboxylic acid cycle in rat hepatocytes.
Hélène A. Buc, C. Augereau, F. Demaugre, A. Moncion +1 more
1983Biochimica et Biophysica Acta8 citations
Semantic Scholar
Clinical presentations of inherited mitochondrial fatty acid oxidation disorders: An update
J. Saudubray, D. Martin, F. Poggi-Travert, T. de Villemeur +11 more
19977 citations
Semantic Scholar
Effects of oxalate and dichloroacetate on lipogenesis and ketogenesis in rat hepatocytes.
Hélène A. Buc, F. Demaugre, A. Moncion, Jean-Paul Leroux
1982Biochemical and Biophysical Research Communications - BBRC7 citations
Semantic Scholar
Contribution of laser microdissection‐based technology to proteomic analysis in hepatocellular carcinoma developing on cirrhosis
A. Dos Santos, V. Thiers, Sokhavuth Sar, N. Dérian +6 more
2007PROTEOMICS - Clinical Applications6 citations Open access
Semantic Scholar
Inborn errors of metabolism in the light of metabolic control analysis.
J. Mazat, Thierry Letellier, M. Malgat, Rodrigue Rossignol +3 more
1998Biochemical Society Transactions6 citations
Semantic Scholar
Pathophysiological approach to carnitine palmitoyltransferase II deficiencies.
F. Demaugre, J. Bonnefont, M. Brivet, C. Cepanec +4 more
1992Progress in clinical and biological research6 citations
Semantic Scholar
The effect of D-carnitine on palmitate oxidation in cultured fibroblasts.
G. Mitchell, J. Saudubray, A. Pellet, F. Demaugre +1 more
1984Clinica chimica acta; international journal of clinical chemistry6 citations
Semantic Scholar
[Heterogeneity of carnitine palmitoyltransferase deficiencies. Deficiency of CPT I in the hepatic form and CPT II in the muscular form].
Jean-Paul Bonnefont, H. Ogier, G. Mitchell, F. Demaugre +3 more
1985Archives francaises de pediatrie4 citations
Semantic Scholar
[Type II carnitine palmitoyl transferase deficiency complicated by acute respiratory failure].
A. Joutel, A. Moulonguet, F. Demaugre, M. Janowski +2 more
1993Revue neurologique (Paris)3 citations
Semantic Scholar
Hepatic and muscular forms of palmitoyl carnitine transferase deficiency.
J. Bonnefont, I. Tein, J. Saudubray, F. Demaugre
1990Progress in clinical and biological research3 citations
Semantic Scholar
Complementation analysis of peroxisomal disorders and classical Refsum.
B. Poll-The, O. Skjeldal, O. Stokke, F. Demaugre +1 more
1990Progress in clinical and biological research2 citations
Semantic Scholar
356 HIP/PAP stimulates liver regeneration by increasing growth associated protein-43 expression through the PKA-dependent phosphorylation of cAMP regulated phosphoprotein-19
H. Lieu, F. Demaugre, M. Simon, J. Girault +4 more
20061 citations Open access
Semantic Scholar
866 IDENTIFICATION OF NOVEL CELLULAR TARGETS IN HUMAN INTRAHEPATIC CHOLANGIOCARCINOMA USING LASER MICRODISSECTION AND AMT-TAG PROTEOMICS
A. Dos Santos, M. Court, V. Thiers, Sokhavuth Sar +6 more
20100 citations
Semantic Scholar
Identification of cellular targets in human intrahepatic cholangiocarcinoma using laser microdissection and AMT-tag proteomics
A. Dos Santos, M. Court, V. Thiers, Sokhavuth Sar +7 more
20100 citations
Semantic Scholar
Identification of cellular targets in human intrahepatic cholangiocarcinoma using laser microdissection and AMT-tag proteomics.
A. Dos Santos, M. Court, V. Thiers, Sokhavuth Sar +7 more
20100 citations
Semantic Scholar
Molecular analysis of hepatic carnitine palmitoy ltransferase 1 deficiency in an extended Hutterite family
C. Prip-Buus, L. Thuillier, N. Abadi, C. Prasad +6 more
20010 citations
Semantic Scholar
Organization of the liver-type human carnitine palmitoyltransferase 1 (CPT1A) gene
S. Gobin, J. Bonnefont, C. Prip-Buus, C. Mugnier +4 more
20010 citations
Semantic Scholar
Role of carnitine palmitoyl transferase II in the oxidation of long chain fatty acids
F. Demaugre, J. Bonnefont, C. Cepanec, J. Saudubray +1 more
19900 citations
Semantic Scholar
[Effects of dichloroacetate and 2-chloropropionate on carbohydrate metabolism of isolated hepatocytes. Therapeutic applications].
J. Leroux, F. Demaugre, H. Buc, J. Saudubray
1980Comptes rendus des seances de la Societe de biologie et de ses filiales0 citations
Semantic Scholar

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