Research Topics
Genomes and GenesSpecies | Eric PasmantSummaryCountry: France Publications
| Collaborators
|
Detail Information
Publications
NF1 microdeletions in neurofibromatosis type 1: from genotype to phenotypeEric Pasmant
UMR745 INSERM, Universite Paris Descartes, Faculte des Sciences Pharmaceutiques et Biologiques, 4 av de l Observatoire, 75006, Paris, France
Hum Mutat 31:E1506-18. 2010..High-resolution array-CGH identified a new recurrent approximately 1.0 Mb microdeletion type, designated as type-3, with breakpoints in the paralogous regions middle NF1-REP-b and distal NF1-REP-c...
Characterization of a 7.6-Mb germline deletion encompassing the NF1 locus and about a hundred genes in an NF1 contiguous gene syndrome patientEric Pasmant
UMR745 INSERM, Universite Paris Descartes, Faculte des Sciences Pharmaceutiques et Biologiques, Paris, France
Eur J Hum Genet 16:1459-66. 2008..This transcript, and/or haploinsufficiency of one or several deleted genes, could explain the clinical severity of the syndrome in this patient...
SPRED1 germline mutations caused a neurofibromatosis type 1 overlapping phenotypeE Pasmant
UMR745 INSERM, Universite Paris Descartes, Faculte des Sciences Pharmaceutiques et Biologiques, Paris, France
J Med Genet 46:425-30. 2009....
Detection and characterization of NF1 microdeletions by custom high resolution array CGHEric Pasmant
UMR745 INSERM, Universite Paris Descartes, Faculte des Sciences Pharmaceutiques et Biologiques, 4 avenue de l Observatoire, 75006 Paris, France
J Mol Diagn 11:524-9. 2009....
Differential expression of CCN1/CYR61, CCN3/NOV, CCN4/WISP1, and CCN5/WISP2 in neurofibromatosis type 1 tumorigenesisEric Pasmant
UMR745 INSERM, Universite Paris Descartes, Faculte des Sciences Pharmaceutiques et Biologiques, Paris, France
J Neuropathol Exp Neurol 69:60-9. 2010..Some of the genes identified, particularly CCN1, might be useful diagnostic or prognostic markers or form the basis for novel therapeutic strategies...
Role of noncoding RNA ANRIL in genesis of plexiform neurofibromas in neurofibromatosis type 1Eric Pasmant
UMR745 INSERM, Universite Paris Descartes, Sorbonne Paris Cité, Faculte des Sciences Pharmaceutiques et Biologiques, 4 avenue de l Observatoire, Paris, France
J Natl Cancer Inst 103:1713-22. 2011..Genetic factors unrelated to the NF1 locus are thought to influence the number of plexiform neurofibromas (PNFs) in patients with NF1, but no factors have been identified to date...
Identification of genes potentially involved in the increased risk of malignancy in NF1-microdeleted patientsEric Pasmant
Universite Paris Descartes, Faculte des Sciences Pharmaceutiques et Biologiques, Paris, France
Mol Med 17:79-87. 2011..This study points to the involvement of several genes (particularly RNF135 and CENTA2) in the increased risk of malignancy observed in NF1-microdeleted patients...
Unravelling the genetic basis of variable clinical expression in neurofibromatosis 1Audrey Sabbagh
Faculte des Sciences Pharmaceutiques et Biologiques, UMR745 INSERM, Universite Paris Descartes, Paris 75006, France
Hum Mol Genet 18:2768-78. 2009..Taken together, our results provided evidence that genetic modifiers, unlinked to the NF1 locus, contribute to the variable expressivity of the disease...
Neurofibromatosis type 1: from genotype to phenotypeEric Pasmant
UMR745 INSERM, Universite Paris Descartes, Sorbonne Paris Cité, Faculte des Sciences Pharmaceutiques et Biologiques, 4 avenue de l Observatoire, Paris 75006, France
J Med Genet 49:483-9. 2012..This phenotypic variability may be due to both modifier genes and environmental factors. Recent targeted strategies have identified several interesting candidate modifier genes...
Two independent de novo mutations as a cause for neurofibromatosis type 1 and Noonan syndrome in a single familyEric Pasmant
Service de Biochimie et de Génétique Moléculaire, Hopital Cochin AP HP, Paris, UMR745 INSERM, Universite Paris Descartes, Sorbonne Paris Cité, Faculte des Sciences Pharmaceutiques et Biologiques, Paris, France
Am J Med Genet A 158:2290-1. 2012..Both de novo mutations were potentially of paternal origin, given the advanced paternal age at the time of conception...
ANRIL, a long, noncoding RNA, is an unexpected major hotspot in GWASEric Pasmant
UMR745 INSERM, Universite Paris Descartes, Faculte des Sciences Pharmaceutiques et Biologiques, 4 avenue de l Observatoire, 75006, Paris, France
FASEB J 25:444-8. 2011..Many GWAS have identified trait-associated SNPs that felt in noncoding genomic regions. It is conceivable to anticipate that long, noncoding RNAs will map to many of these "gene deserts."..
Different sized somatic NF1 locus rearrangements in neurofibromatosis 1-associated malignant peripheral nerve sheath tumorsEric Pasmant
UMR745 INSERM, Universite Paris Descartes, Faculte des Sciences Pharmaceutiques et Biologiques, 75006 Paris, France
J Neurooncol 102:341-6. 2011..As NF1 patients with such germline NF1 deletions do exhibit increased risk of developing MPNST, these present findings emphasize the likely role of at least some of these NF1 flanking genes in MPNST pathobiology...
Somatic NF1 inactivation is a frequent event in sporadic pheochromocytomaNelly Burnichon
INSERM, UMR970, Paris Cardiovascular Research Center, Paris, France
Hum Mol Genet 21:5397-405. 2012..These new findings suggest that NF1 loss of function is a frequent event in the tumorigenesis of sporadic pheochromocytoma and strengthen the new concept of molecular-based targeted therapy for pheochromocytoma or paraganglioma...
Cardio-facio-cutaneous and Noonan syndromes due to mutations in the RAS/MAPK signalling pathway: genotype-phenotype relationships and overlap with Costello syndromeCaroline Nava
Department of Genetics, AP HP, Hopital Robert Debre, Paris, France
J Med Genet 44:763-71. 2007..Although some associated features appear to be characteristic of a specific gene, no simple rule exists to distinguish NS from CFC easily...
First description of ABCB4 gene deletions in familial low phospholipid-associated cholelithiasis and oral contraceptives-induced cholestasisEric Pasmant
Service de Biochimie et de Génétique Moléculaire, Hopital Beaujon, Clichy, France
Eur J Hum Genet 20:277-82. 2012..Further comparative studies of patients with well-characterized genotypes (including deletions) and phenotypes will help determine whether ABCB4 mutation types influence clinical outcomes...
