Claudine Junien

Heterozygous TGFBR2 mutations in Marfan syndrome. (2007)

Mizuguchi, Takeshi, Collod-Beroud, Gwenaëlle, Akiyama, Takushi, Abifadel, Marianne, Harada, Naoki, Morisaki, Takayuki, ...

Marfan syndrome is an extracellular matrix disorder with cardinal manifestations in the eye, skeleton and cardiovascular systems associated with defects in the gene encoding fibrillin (FBN1) at...

Heterozygous TGFBR2 mutations in Marfan syndrome. (2007)

Mizuguchi, Takeshi, Collod-Beroud, Gwenaëlle, Akiyama, Takushi, Abifadel, Marianne, Harada, Naoki, Morisaki, Takayuki, ...

Marfan syndrome is an extracellular matrix disorder with cardinal manifestations in the eye, skeleton and cardiovascular systems associated with defects in the gene encoding fibrillin (FBN1) at...

A second locus for Marfan syndrome maps to chromosome 3p24.2-p25. (2007)

Collod, Gwenaëlle, Babron, Marie-Claude, Jondeau, Guillaume, Coulon, Monique, Weissenbach, Jean, Dubourg, Olivier, ...

Marfan syndrome (MFS) is an autosomal dominant connective-tissue disorder characterized by skeletal, ocular and cardiovascular defects of highly variable expressivity. The diagnosis relies solely on...

A second locus for Marfan syndrome maps to chromosome 3p24.2-p25. (2007)

Collod, Gwenaëlle, Babron, Marie-Claude, Jondeau, Guillaume, Coulon, Monique, Weissenbach, Jean, Dubourg, Olivier, ...

Marfan syndrome (MFS) is an autosomal dominant connective-tissue disorder characterized by skeletal, ocular and cardiovascular defects of highly variable expressivity. The diagnosis relies solely on...

CTG Trinucleotide Repeat “Big Jumps”: Large Expansions, Small Mice (2007)

Mário Gomes-Pereira, Laurent Foiry, Annie Nicole, Aline Huguet, Claudine Junien, Arnold Munnich, ...

Trinucleotide repeat expansions are the genetic cause of numerous human diseases, including fragile X mental retardation, Huntington disease, and myotonic dystrophy type 1. Disease severity and age...

CTG trinucleotide repeat "big jumps": large expansions, small mice (2007)

Mário Gomes-Pereira, Laurent Foiry, Annie Nicole, Aline Huguet, Claudine Junien, Arnold Munnich, ...

Trinucleotide repeat expansions are the genetic cause of numerous human diseases, including fragile X mental retardation, Huntington disease and myotonic dystrophy type 1. Disease severity and...

Épigénomique nutritionnelle du syndrome métabolique (2006)

Junien, Claudine, Gallou-Kabani, Catherine, Vigé, Alexandre, Gross, Marie-Sylvie

The importance of epigenetic alterations has been acknowledged in cancer for about two decades by an increasing number of molecular oncologists who contributed to deciphering the epigenetic codes and...

Épigénomique nutritionnelle du syndrome métabolique (2006)

Junien, Claudine, Gallou-Kabani, Catherine, Vigé, Alexandre, Gross, Marie-Sylvie

The importance of epigenetic alterations has been acknowledged in cancer for about two decades by an increasing number of molecular oncologists who contributed to deciphering the epigenetic codes and...

Épigénomique nutritionnelle du syndrome métabolique (2006)

Junien, Claudine, Gallou-Kabani, Catherine, Vigé, Alexandre, Gross, Marie-Sylvie

The importance of epigenetic alterations has been acknowledged in cancer for about two decades by an increasing number of molecular oncologists who contributed to deciphering the epigenetic codes and...

Épigénomique nutritionnelle du syndrome métabolique (2006)

Junien, Claudine, Gallou-Kabani, Catherine, Vigé, Alexandre, Gross, Marie-Sylvie

The importance of epigenetic alterations has been acknowledged in cancer for about two decades by an increasing number of molecular oncologists who contributed to deciphering the epigenetic codes and...

The case for strategic international alliances to harness nutritional genomics for public and personal health (2005)

Kaput, Jim, Ordovas, José, Ferguson, Lynnette, Van Ommen, Ben, Rodríguez, Raymond, Allen, Lindsay, ...

Nutrigenomics is the study of how constituents of the diet interact with genes, and their products, to alter phenotype and, conversely, how genes and their products metabolise these constituents into...

Épigénomique nutritionnelle du syndrome métabolique (2005)

Junien, Claudine, Gallou-Kabani, Catherine, Vigé, Alexandre, Gross, Marie-Sylvie

The importance of epigenetic alterations has been acknowledged in cancer for about two decades by an increasing number of molecular oncologists who contributed to deciphering the epigenetic codes and...

Tabac : comprendre la dépendance pour agir (2004)

Abrous, Nora, Aubin, Henri Jean, Berlin, Ivan, Junien, Claudine, Kaminski, Monique, Le Foll, Bernard, ...

473 pages, tableaux, graphiques, figures, références bibliographiques disséminées

LDLR Database (second edition): new additions to the database and the software, and results of the first molecular analysis (1997)

Mathilde Varret, Rochelle Thiart, Maritha J. Kotze, Heike Baron, Ana Cenarro, Olivier Descamps, ...

Mutations in the LDL receptor gene (LDLR) cause familial hypercholesterolemia (FH), a common autosomal dominant disorder. The LDLR database is a computerized tool that has been developed to provide...

Marfan Database (third edition): new mutations and new routines for the software (1997)

Lesley Ades, Cheryl Black, Maureen Boxer, Katherine J. Holman, Anne Paepe, ...

The Marfan database is a software that contains routines for the analysis of mutations identified in the FBN1 gene that encodes fibrillin-1. Mutations in this gene are associated not only with Marfan...

CTG repeat instability and size variation timing in DNA repair-deficient mice

Savouret, Cédric, Brisson, Edith, Essers, Jeroen, Kanaar, Roland, Pastink, Albert, Te Riele, Hein, ...

Type 1 myotonic dystrophy is caused by the expansion of an unstable CTG repeat in the DMPK gene. We have investigated the molecular mechanisms underlying the CTG repeat instability by crossing...

MSH2-Dependent Germinal CTG Repeat Expansions Are Produced Continuously in Spermatogonia from DM1 Transgenic Mice

Savouret, Cédric, Garcia-Cordier, Corinne, Megret, Jérôme, Te Riele, Hein, Junien, Claudine, Gourdon, Geneviève

Myotonic dystrophy type 1 is a neuromuscular affection associated with the expansion of an unstable CTG repeat in the DM protein kinase gene. The disease is characterized by somatic tissue-specific...

Autosomal dominant Marfan-like connective-tissue disorder with aortic dilation and skeletal anomalies not linked to the fibrillin genes

Boileau, Catherine, Jondeau, Guillaume, Babron, Marie-Claude, Coulon, Monique, Alexandre, Jeanne-Armelle, Sakai, Lynn, ...

We describe a large family with a connective-tissue disorder that exhibits some of the skeletal and cardiovascular features seen in Marfan syndrome. However, none of the 19 affected individuals...

CTG repeat instability and size variation timing in DNA repair-deficient mice

Savouret, Cédric, Brisson, Edith, Essers, Jeroen, Kanaar, Roland, Pastink, Albert, Te Riele, Hein, ...

Type 1 myotonic dystrophy is caused by the expansion of an unstable CTG repeat in the DMPK gene. We have investigated the molecular mechanisms underlying the CTG repeat instability by crossing...

MSH2-Dependent Germinal CTG Repeat Expansions Are Produced Continuously in Spermatogonia from DM1 Transgenic Mice

Savouret, Cédric, Garcia-Cordier, Corinne, Megret, Jérôme, Te Riele, Hein, Junien, Claudine, Gourdon, Geneviève

Myotonic dystrophy type 1 is a neuromuscular affection associated with the expansion of an unstable CTG repeat in the DM protein kinase gene. The disease is characterized by somatic tissue-specific...

Autosomal dominant Marfan-like connective-tissue disorder with aortic dilation and skeletal anomalies not linked to the fibrillin genes

Boileau, Catherine, Jondeau, Guillaume, Babron, Marie-Claude, Coulon, Monique, Alexandre, Jeanne-Armelle, Sakai, Lynn, ...

We describe a large family with a connective-tissue disorder that exhibits some of the skeletal and cardiovascular features seen in Marfan syndrome. However, none of the 19 affected individuals...

CTG Trinucleotide Repeat “Big Jumps”: Large Expansions, Small Mice

Gomes-Pereira, Mário, Foiry, Laurent, Nicole, Annie, Huguet, Aline, Junien, Claudine, Munnich, Arnold, ...

Trinucleotide repeat expansions are the genetic cause of numerous human diseases, including fragile X mental retardation, Huntington disease, and myotonic dystrophy type 1. Disease severity and age...

Unbalanced Expression of 11p15 Imprinted Genes in Focal Forms of Congenital Hyperinsulinism : Association with a Reduction to Homozygosity of a Mutation in ABCC8 or KCNJ11

Fournet, Jean-Christophe, Mayaud, Christine, De Lonlay, Pascale, Gross-Morand, Marie-Sylvie, Verkarre, Virginie, Castanet, Mireille, ...

Congenital hyperinsulinism (CHI), previously named persistent hyperinsulinemic hypoglycemia of infancy, is characterized by profound hypoglycemia because of excessive insulin secretion. CHI presents...