O
Olivier Didierjean
Researcher at French Institute of Health and Medical Research
Publications - 17
Citations - 2870
Olivier Didierjean is an academic researcher from French Institute of Health and Medical Research. The author has contributed to research in topics: Spinocerebellar ataxia & Machado–Joseph disease. The author has an hindex of 16, co-authored 17 publications receiving 2763 citations. Previous affiliations of Olivier Didierjean include Harvard University.
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Journal ArticleDOI
Cloning of the gene for spinocerebellar ataxia 2 reveals a locus with high sensitivity to expanded CAG/glutamine repeats
Georges Imbert,Frédéric Saudou,Frédéric Saudou,Gaël Yvert,Gaël Yvert,Didier Devys,Didier Devys,Yvon Trottier,Yvon Trottier,Jean-Marie Garnier,Jean-Marie Garnier,Chantal Weber,Chantal Weber,Jean-Louis Mandel,Jean-Louis Mandel,Géraldine Cancel,Géraldine Cancel,Nacer Abbas,Nacer Abbas,Alexandra Durr,Alexandra Durr,Olivier Didierjean,Olivier Didierjean,Giovanni Stevanin,Giovanni Stevanin,Yves Agid,Yves Agid,Alexis Brice,Alexis Brice +28 more
TL;DR: The steep inverse correlation between age of onset and CAG number suggests a higher sensitivity to polyglutamine length than in the other polyglUTamine expansion diseases.
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Spinocerebellar ataxia 3 and Machado-Joseph disease: clinical, molecular, and neuropathological features
Alexandra Durr,Giovanni Stevanin,Géraldine Cancel,Charles Duyckaerts,Nacer Abbas,Olivier Didierjean,Hervé Chneiweiss,Ali Benomar,Olivier Lyon-Caen,Jean Julien,M Serdaru,C. Penet,Yves Agid,Alexis Brice +13 more
TL;DR: The length of the CAG repeat influenced the frequency of clinical signs associated with cerebellar ataxia, such as abnormal tendon reflexes or decreased vibration sense, whereas the interindividual variation of supranuclear ophthalmoplegia, sphincter and swallowing difficulties, and amyotrophy was mostly determined by different disease durations.
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Mutations of the presenilin I gene in families with early-onset Alzheimer's disease.
Dominique Campion,Dominique Campion,Jean-Michel Flaman,Alexis Brice,Didier Hannequin,Bruno Dubois,Cosette Martin,Viviane Moreau,Françoise Charbonnier,Olivier Didierjean,S. Tardieu,C. Penet,Michèle Puel,Florence Pasquier,François Le Doze,Gil Bellis,Alphonse Calenda,Roland Heilig,Maria Martinez,Jacques Mallet,Michel Bellis,Françoise Clerget-Darpoux,Yves Agid,Thierry Frebourg +23 more
TL;DR: The results support the notion that PSNLI is the major gene involved in autosomal dominant EOAD, and underlines the great allelic heterogeneity and the large distribution of the mutations within thePSNLI coding region.
Journal ArticleDOI
Molecular and Clinical Correlations in Spinocerebellar Ataxia 2: A Study of 32 Families
Géraldine Cancel,Alexandra Durr,Olivier Didierjean,Georges Imbert,Katrin Bürk,Agnès Lézin,Samir Belal,Ali Benomar,Myriem Abada-Bendib,Christophe Vial,João Guimarães,Hervé Chneiweiss,Giovanni Stevanin,Gaël Yvert,Nacer Abbas,Frédéric Saudou,Anne-Sophie Lebre,Mohamed Yahyaoui,Fayçal Hentati,Jean-Claude Vernant,Thomas Klockgether,Jean-Louis Mandel,Yves Agid,Alexis Brice +23 more
TL;DR: The frequency of several clinical signs such as myoclonus, dystonia and myokymia increased with the number of CAG repeats whereas the frequency of others was related to disease duration, and instability was confirmed by the high degree of gonadal mosaicism observed in sperm DNA of one patient.
Journal ArticleDOI
Autosomal dominant cerebellar ataxia type I Clinical features and MRI in families with SCA1, SCA2 and SCA3
Katrin Bürk,Michael Abele,Michael Fetter,Johannes Dichgans,Martin Skalej,Franco Laccone,Olivier Didierjean,Alexis Brice,Thomas Klockgether +8 more
TL;DR: The principal finding of this study is that mutations of the SCA2 and SCA3 gene cause phenotypes which can be distinguished in vivo by recording of eye movements and morphometric MRI analysis.