J
Jean-Louis Mandel
Researcher at University of Strasbourg
Publications - 372
Citations - 39716
Jean-Louis Mandel is an academic researcher from University of Strasbourg. The author has contributed to research in topics: Gene & Fragile X syndrome. The author has an hindex of 101, co-authored 362 publications receiving 37522 citations. Previous affiliations of Jean-Louis Mandel include French Institute of Health and Medical Research & Collège de France.
Papers
More filters
Journal ArticleDOI
α-Amanitin: A specific inhibitor of one of two DNA-dependent RNA polymerase activities from calf thymus
Claude Kedinger,Claude Kedinger,M. Gniazdowski,M. Gniazdowski,Jean-Louis Mandel,Jean-Louis Mandel,F. Gissinger,F. Gissinger,Pierre Chambon,Pierre Chambon +9 more
TL;DR: α-amanitin selectively inhibits RNA synthesis catalyzed by calf thymus RNA polymerase activity B by interacting with the enzyme and inhibiting chain elongation as discussed by the authors, which can be used to identify the source of RNA synthesis.
Journal ArticleDOI
Adenovirus as an expression vector in muscle cells in vivo
TL;DR: A recombinant adenovirus is constructed containing the beta-galactosidase reporter gene under the control of muscle-specific regulatory sequences and it is reported that its in vivo expression up to 75 days after infection compares very favorably with other strategies proposed for gene or myoblast transfer in muscle in vivo.
Journal ArticleDOI
Proteases acting on mutant huntingtin generate cleaved products that differentially build up cytoplasmic and nuclear inclusions.
Astrid Lunkes,Katrin S. Lindenberg,Léa Ben-Haïem,Chantal Weber,Didier Devys,G. Bernhard Landwehrmeyer,Jean-Louis Mandel,Yvon Trottier +7 more
TL;DR: It is reported that two distinct mhtt fragments, termed cp-A and cp-B, differentially build up nuclear and cytoplasmic inclusions in HD brain and in a cellular model for HD.
Journal ArticleDOI
Mutations in the kinase Rsk-2 associated with Coffin-Lowry syndrome.
Elisabeth Trivier,Dario De Cesare,Sylvie Jacquot,Solange Pannetier,Elaine H. Zackai,Ian Young,Jean-Louis Mandel,Paolo Sassone-Corsi,André Hanauer +8 more
TL;DR: Findings provide direct evidence that abnormalities in the MAPK/RSK signalling pathway cause Coffin-Lowry syndrome.
Journal ArticleDOI
Fragile X syndrome
Randi J Hagerman,Elizabeth Berry-Kravis,Heather C. Hazlett,Donald B. Bailey,Hervé Moine,R. Frank Kooy,Flora Tassone,Ilse Gantois,Nahum Sonenberg,Jean-Louis Mandel,Paul J. Hagerman +10 more
TL;DR: Fragile X syndrome (FXS) is the leading inherited form of intellectual disability and autism spectrum disorder, and patients can present with severe behavioural alterations, including hyperactivity, impulsivity and anxiety, in addition to poor language development and seizures as mentioned in this paper.