1. B. O. Ben Cheikh, S. Baulac, F. Lahjouji, A. Bouhouche, P. Couarch, N. Khalili, W. Regragui, S. Lehericy, M. Ruberg, A. Benomar, S. Heath, T. Chkili, M. Yahyaoui, M. Jiddane, R. Ouazzani, and E. LeGuern. A locus for bilateral occipital polymicrogyria maps to chromosome 6q16-q22. Neurogenetics, 10(1):35-42, 2009. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Adult, Chromosome Mapping, *Chromosomes, Human, Pair 6, Consanguinity, DNA Mutational Analysis, Family, Female, Genetic Predisposition to Disease, Humans, Lod Score, Magnetic Resonance Imaging, Male, Malformations of Cortical Development/*genetics/pathology/physiopathology, Middle Aged, Morocco, Polymorphism, Single Nucleotide, Young Adult.



2. C. Depienne, D. Bouteiller, B. Keren, E. Cheuret, K. Poirier, O. Trouillard, B. Benyahia, C. Quelin, W. Carpentier, S. Julia, A. Afenjar, A. Gautier, F. Rivier, S. Meyer, P. Berquin, M. Helias, I. Py, S. Rivera, N. Bahi-Buisson, I. Gourfinkel-An, C. Cazeneuve, M. Ruberg, A. Brice, R. Nabbout, and E. Leguern. Erratum. PLoS Genet, 5(4), 2009. Note: Journal ArticleUnited States. [WWW]



3. C. Depienne, D. Bouteiller, B. Keren, E. Cheuret, K. Poirier, O. Trouillard, B. Benyahia, C. Quelin, W. Carpentier, S. Julia, A. Afenjar, A. Gautier, F. Rivier, S. Meyer, P. Berquin, M. Helias, I. Py, S. Rivera, N. Bahi-Buisson, I. Gourfinkel-An, C. Cazeneuve, M. Ruberg, A. Brice, R. Nabbout, and E. Leguern. Sporadic infantile epileptic encephalopathy caused by mutations in PCDH19 resembles Dravet syndrome but mainly affects females. PLoS Genet, 5(2):e1000381, 2009. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW]



4. M. Hollerhage, A. Matusch, P. Champy, A. Lombes, M. Ruberg, W. H. Oertel, and G. U. Hoglinger. Natural lipophilic inhibitors of mitochondrial complex I are candidate toxins for sporadic neurodegenerative tau pathologies. Exp Neurol, 2009. Note: Journal article. [WWW]



5. M. P. Muriel, A. Dauphin, M. Namekawa, A. Gervais, A. Brice, and M. Ruberg. Atlastin-1, the dynamin-like GTPase responsible for spastic paraplegia SPG3A, remodels lipid membranes and may form tubules and vesicles in the endoplasmic reticulum. J Neurochem, 110(5):1607-16, 2009. Note: Comparative StudyJournal ArticleResearch Support, Non-U.S. Gov'tEngland. [WWW] Keyword(s): Cell Line, Cytoplasmic Vesicles/*enzymology/genetics/ultrastructure, Dynamins/genetics/metabolism, Endoplasmic Reticulum/*enzymology/genetics/ultrastructure, GTP Phosphohydrolases/genetics/*metabolism/physiology, Humans, Membrane Lipids/genetics/*metabolism, Microtubules/*enzymology/genetics/ultrastructure, Protein Transport/physiology, Spastic Paraplegia, Hereditary/enzymology/genetics.



6. A. Camuzat, M. Romana, A. Durr, J. Feingold, A. Brice, M. Ruberg, and A. Lannuzel. The PSP-associated MAPT H1 subhaplotype in Guadeloupean atypical parkinsonism. Mov Disord, 23(16):2384-91, 2008. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited Statesofficial journal of the Movement Disorder Society. [WWW] Keyword(s): Aged, Case-Control Studies, Chi-Square Distribution, European Continental Ancestry Group, Female, Gene Frequency, Genotype, Guadeloupe/epidemiology, Humans, Linkage Disequilibrium, Male, Middle Aged, Parkinsonian Disorders/complications/*genetics, Polymorphism, Single Nucleotide/*genetics, Supranuclear Palsy, Progressive/complications/*genetics, tau Proteins/*classification/*genetics.



7. A. Lannuzel, M. Ruberg, and P. P. Michel. Atypical parkinsonism in the Caribbean island of Guadeloupe: etiological role of the mitochondrial complex I inhibitor annonacin. Mov Disord, 23(15):2122-8, 2008. Note: Journal ArticleResearch Support, Non-U.S. Gov'tReviewUnited Statesofficial journal of the Movement Disorder Society. [WWW] Keyword(s): Animals, Electron Transport Complex I/*antagonists & inhibitors, Furans/*administration & dosage, Guadeloupe/epidemiology, Humans, Lactones/*administration & dosage, Mitochondria/*drug effects/metabolism, Parkinsonian Disorders/epidemiology/*etiology/*pathology.



8. B. Ouled Amar Ben Cheikh, S. Baulac, F. Lahjouji, A. Bouhouche, P. Couarch, N. Khalili, W. Regragui, S. Lehericy, M. Ruberg, A. Benomar, S. Heath, T. Chkili, M. Yahyaoui, M. Jiddane, R. Ouazzani, and E. Leguern. A locus for bilateral occipital polymicrogyria maps to chromosome 6q16-q22. Neurogenetics, 2008. Note: Journal article. [WWW]



9. C. Saint-Martin, D. Bouteiller, G. Stevanin, C. Popescu, C. Charon, M. Ruberg, S. Baulac, E. Leguern, P. Labauge, and C. Depienne. Refinement of the 2p11.1-q12.2 locus responsible for cortical tremor associated with epilepsy and exclusion of candidate genes. Neurogenetics, 9(1):69-71, 2008. Note: Journal article. [WWW]



10. G. Stevanin, M. Ruberg, and A. Brice. Recent advances in the genetics of spastic paraplegias. Curr Neurol Neurosci Rep, 8(3):198-210, 2008. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW]



11. S. T. du Montcel, P. Charles, P. Ribai, C. Goizet, A. Le Bayon, P. Labauge, L. Guyant-Marechal, S. Forlani, C. Jauffret, N. Vandenberghe, K. N'Guyen, I. Le Ber, D. Devos, C. M. Vincitorio, M. U. Manto, F. Tison, D. Hannequin, M. Ruberg, A. Brice, and A. Durr. Composite cerebellar functional severity score: validation of a quantitative score of cerebellar impairment. Brain, 131(Pt 5):1352-61, 2008. Note: Journal ArticleMulticenter StudyResearch Support, Non-U.S. Gov'tValidation StudiesEnglanda journal of neurology. [WWW] Keyword(s): Adult, Aged, Cerebellar Ataxia/*physiopathology/psychology, Female, Humans, Male, Middle Aged, Neuropsychological Tests, Paraplegia/*physiopathology/psychology, Psychometrics, Psychomotor Disorders/*etiology, Quality of Life, *Severity of Illness Index.



12. A. Bouhouche, N. Birouk, H. Azzedine, A. Benomar, G. Durosier, D. Ente, M. P. Muriel, M. Ruberg, I. Slassi, M. Yahyaoui, O. Dubourg, R. Ouazzani, and E. LeGuern. Autosomal recessive axonal Charcot-Marie-Tooth disease (ARCMT2): phenotype-genotype correlations in 13 Moroccan families. Brain, 130:1062-1075, 2007. Note: Part 4. [WWW]



13. A. Chojnowski, N. Ravise, C. Bachelin, C. Depienne, M. Ruberg, B. Brugg, J. Laporte, A. Baron-Van Evercooren, and E. LeGuern. Silencing of the Charcot-Marie-Tooth associated MTMR2 gene decreases proliferation and enhances cell death in primary cultures of Schwann cells. Neurobiol Dis, 26(2):323-31, 2007. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Animals, Animals, Newborn, Apoptosis/drug effects/genetics, Caspases/metabolism, Cell Death/drug effects/genetics, Cell Proliferation, Cells, Cultured, Charcot-Marie-Tooth Disease/*genetics/metabolism/physiopathology, Culture Media, Serum-Free/pharmacology, Down-Regulation/drug effects/genetics, Gene Expression Profiling, Gene Expression Regulation, Developmental/genetics, *Gene Silencing, Myelin Sheath/*genetics/metabolism/pathology, Peripheral Nerves/*metabolism/pathology/physiopathology, Protein Tyrosine Phosphatases/*genetics, Protein Tyrosine Phosphatases, Non-Receptor, RNA Interference, Rats, Schwann Cells/*metabolism/pathology.



14. V. C. De Cock, A. Lannuzel, S. Verhaeghe, E. Roze, M. Ruberg, J. P. Derenne, J. C. Willer, M. Vidailhet, and I. Arnulf. REM sleep behavior disorder in patients with guadeloupean parkinsonism, a tauopathy. Sleep, 30(8):1026-32, 2007. Note: Journal ArticleUnited States. [WWW] Keyword(s): Aged, Dementia/chemically induced/diagnosis, Diagnosis, Differential, Disability Evaluation, Dreams/drug effects, Fatty Alcohols/*toxicity, Female, Fruit/*toxicity, Guadeloupe, Humans, Lactones/*toxicity, Male, Mesencephalon/drug effects, Middle Aged, Motor Activity/drug effects, Neurologic Examination/drug effects, Parkinson Disease/diagnosis, Parkinsonian Disorders/*chemically induced/diagnosis, Polysomnography/drug effects, Prospective Studies, REM Sleep Behavior Disorder/*chemically induced/diagnosis, Sleep-Wake Transition Disorders/chemically induced/diagnosis, Supranuclear Palsy, Progressive/chemically induced/diagnosis, Tauopathies/*chemically induced/diagnosis.



15. C. Depienne, E. Fedirko, J. M. Faucheux, S. Forlani, B. Bricka, C. Goizet, S. Lesourd, G. Stevanin, M. Ruberg, A. Durr, and A. Brice. A de novo SPAST mutation leading to somatic mosaicism is associated with a later age at onset in HSP. Neurogenetics, 8(3):231-233, 2007. Note: Journal article. [WWW]



16. C. Depienne, E. Fedirko, S. Forlani, C. Cazeneuve, P. Ribai, I. Feki, C. Tallaksen, K. Nguyen, B. Stankoff, M. Ruberg, G. Stevanin, A. Durr, and A. Brice. Exon deletions of SPG4 are a frequent cause of hereditary spastic paraplegia. J Med Genet, 44(4):281-4, 2007. Note: Comparative StudyLetterResearch Support, Non-U.S. Gov'tEngland. [WWW] Keyword(s): Adenosine Triphosphatases/deficiency/*genetics, Adolescent, Adult, Age of Onset, Aged, Child, Child, Preschool, DNA Mutational Analysis, Exons/*genetics, Female, France/epidemiology, Genetic Heterogeneity, Genetic Screening, Humans, Infant, Male, Middle Aged, Point Mutation, Polymerase Chain Reaction/methods, Portugal/epidemiology, Spain/epidemiology, Spastic Paraplegia, Hereditary/epidemiology/*genetics.



17. M. Escobar-Khondiker, M. Hollerhage, M. P. Muriel, P. Champy, A. Bach, C. Depienne, G. Respondek, E. S. Yamada, A. Lannuzel, T. Yagi, E. C. Hirsch, W. H. Oertel, R. Jacob, P. P. Michel, M. Ruberg, and G. U. Hoglinger. Annonacin, a natural mitochondrial complex I inhibitor, causes tau pathology in cultured neurons. J Neurosci, 27(29):7827-37, 2007. Note: Dk053244/dk/niddkNs048441/ns/nindsJournal ArticleResearch Support, N.I.H., ExtramuralResearch Support, Non-U.S. Gov'tUnited Statesthe official journal of the Society for Neuroscience. [WWW] Keyword(s): Adenosine Triphosphate/metabolism, Animals, Brain/cytology, Cell Death/drug effects, Cell Survival/drug effects, Cells, Cultured, Dose-Response Relationship, Drug, Embryo, Enzyme Inhibitors/*pharmacology, Female, Furans/*pharmacology, Lactones/*pharmacology, Microscopy, Immunoelectron/methods, Microtubule-Associated Proteins/metabolism, Neurons/*drug effects/metabolism/ultrastructure, Paclitaxel/pharmacology, Pregnancy, Rats, Rats, Wistar, Reactive Oxygen Species/metabolism, Tubulin Modulators/pharmacology, tau Proteins/*metabolism.



18. A. Lannuzel, G. U. Hoglinger, S. Verhaeghe, L. Gire, S. Belson, M. Escobar-Khondiker, P. Poullain, W. H. Oertel, E. C. Hirsch, B. Dubois, and M. Ruberg. Atypical parkinsonism in Guadeloupe: a common risk factor for two closely related phenotypes?. Brain, 130:816-827, 2007. Note: Part 3. [WWW]



19. Morwena Latouche, Christelle Lasbleiz, Elodie Martin, Veronique Monnier, Thomas Debeir, Annick Mouatt-Prigent, Marie-Paule Muriel, Lydie Morel, Merle Ruberg, Alexis Brice, Giovanni Stevanin, and Herve Tricoire. A Conditional Pan-Neuronal Drosophila Model of Spinocerebellar Ataxia 7 with a Reversible Adult Phenotype Suitable for Identifying Modifier Genes. J Neurosci, 27(10):2483-2492, 2007. [WWW] [doi:10.1523/jneurosci.5453-06.2007]



20. M. Latouche, C. Lasbleiz, E. Martin, V. Monnier, T. Debeir, A. Mouatt-Prigent, M. P. Muriel, L. Morel, M. Ruberg, A. Brice, G. Stevanin, and H. Tricoire. A conditional pan-neuronal Drosophila model of spinocerebellar ataxia 7 with a reversible adult phenotype suitable for identifying modifier genes. J Neurosci, 27(10):2483-92, 2007. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited Statesthe official journal of the Society for Neuroscience. [WWW] Keyword(s): Animals, Animals, Genetically Modified, Cell Death, Cells, Cultured, *Disease Models, Animal, *Drosophila, Dyskinesias/genetics, Glutamine, Humans, Intranuclear Inclusion Bodies/ultrastructure, Longevity, Male, Mutation, Nerve Tissue Proteins/genetics/metabolism, Neurons/metabolism, Peptides/genetics, Phenotype, Rats, Spinocerebellar Ataxias/*genetics/metabolism/pathology/physiopathology, Threonine.



21. S. Lesage, L. Leclere, E. Lohmann, M. Borg, M. Ruberg, A. Durr, and A. Brice. Frequency of the LRRK2 G2019S mutation in siblings with Parkinson's disease. Neurodegener Dis, 4(2-3):195-8, 2007. Note: French Parkinson's Disease Genetics Study GroupNs41723-01a1/ns/nindsJournal ArticleResearch Support, N.I.H., ExtramuralResearch Support, Non-U.S. Gov'tSwitzerland. [WWW]



22. L. Mallet, M. Schupbach, K. N'Diaye, P. Remy, E. Bardinet, V. Czernecki, M. L. Welter, A. Pelissolo, M. Ruberg, Y. Agid, and J. Yelnik. Stimulation of subterritories of the subthalamic nucleus reveals its role in the integration of the emotional and motor aspects of behavior. Proc Natl Acad Sci U S A, 104(25):10661-6, 2007. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW]



23. R. Nabbout, S. Baulac, I. Desguerre, N. Bahi-Buisson, C. Chiron, M. Ruberg, O. Dulac, and E. LeGuern. New locus for febrile seizures with absence epilepsy on 3p and a possible modifier gene on 18p. Neurology, 68(17):1374-81, 2007. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Child, Preschool, Chromosomes, Human, Pair 18/*genetics, Chromosomes, Human, Pair 3/*genetics, Electroencephalography, Epilepsy, Absence/*genetics, Epilepsy, Temporal Lobe/genetics, Epilepsy, Tonic-Clonic/genetics, *Epistasis, Genetic, Female, France, Genes, Genetic Markers, Genotype, Haplotypes, Hippocampus/pathology, Humans, Infant, Magnetic Resonance Imaging, Male, Pedigree, Penetrance, Phenotype, Sclerosis/pathology, Seizures, Febrile/*genetics.



24. M. Namekawa, M. P. Muriel, A. Janer, M. Latouche, A. Dauphin, T. Debeir, E. Martin, C. Duyckaerts, A. Prigent, C. Depienne, A. Sittler, A. Brice, and M. Ruberg. Mutations in the SPG3A gene encoding the GTPase atlastin interfere with vesicle trafficking in the ER/Golgi interface and Golgi morphogenesis. Mol Cell Neurosci, 35(1):1-13, 2007. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Adult, Cell Line, Cytoplasmic Vesicles/*enzymology/ultrastructure, Endoplasmic Reticulum/*enzymology/ultrastructure, Epitopes, GTP Phosphohydrolases/*genetics/*metabolism, Golgi Apparatus/*enzymology/ultrastructure, Humans, Kidney/cytology, Microscopy, Electron, Motor Cortex/cytology, Motor Neurons/*enzymology, Paraplegia/genetics/metabolism, Point Mutation, Protein Transport/physiology, Spinal Cord/cytology.



25. A. S. Rolland, M. T. Herrero, V. Garcia-Martinez, M. Ruberg, E. C. Hirsch, and C. Francois. Metabolic activity of cerebellar and basal ganglia-thalamic neurons is reduced in parkinsonism. Brain, 130(Pt 1):265-75, 2007. Note: Journal ArticleResearch Support, Non-U.S. Gov'tEnglanda journal of neurology. [WWW] Keyword(s): Animals, Basal Ganglia/*metabolism, Cerebellum/*metabolism, Corpus Striatum/enzymology, Disease Models, Animal, Dopamine/metabolism, Dopamine Agents, Electron Transport Complex IV/analysis, Haplorhini, Immunohistochemistry/methods, In Situ Hybridization/methods, Interneurons/metabolism, Neural Pathways/metabolism, Neurons/*metabolism, Oxidopamine, Parkinson Disease/*metabolism, RNA, Messenger/analysis, Rats, Substantia Nigra/enzymology, Sympatholytics, Thalamus/*metabolism, Tyrosine 3-Monooxygenase/analysis.



26. G. Stevanin, F. M. Santorelli, H. Azzedine, P. Coutinho, J. Chomilier, P. S. Denora, E. Martin, A. M. Ouvrard-Hernandez, A. Tessa, N. Bouslam, A. Lossos, P. Charles, J. L. Loureiro, N. Elleuch, C. Confavreux, V. T. Cruz, M. Ruberg, E. Leguern, D. Grid, M. Tazir, B. Fontaine, A. Filla, E. Bertini, A. Durr, and A. Brice. Mutations in SPG11, encoding spatacsin, are a major cause of spastic paraplegia with thin corpus callosum. Nat Genet, 39(3):366-372, 2007. Note: Journal article. [WWW]



27. H. Azzedine, N. Ravise, C. Verny, A. Gabreels-Festen, M. Lammens, D. Grid, J. M. Vallat, G. Durosier, J. Senderek, S. Nouioua, T. Hamadouche, A. Bouhouche, A. Guilbot, C. Stendel, M. Ruberg, A. Brice, N. Birouk, O. Dubourg, M. Tazir, and E. LeGuern. Spine deformities in Charcot-Marie-Tooth 4C caused by SH3TC2 gene mutations. Neurology, 67(4):602-6, 2006. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Charcot-Marie-Tooth Disease/*epidemiology/*genetics, Chromosome Mapping, DNA Mutational Analysis, Female, France/epidemiology, Genetic Predisposition to Disease/epidemiology/genetics, Humans, Incidence, Male, Mutation, Pedigree, Risk Assessment/*methods, Risk Factors, Spinal Curvatures/*epidemiology/*genetics, Spine/*abnormalities.



28. C. Depienne, A. Arzimanoglou, O. Trouillard, E. Fedirko, S. Baulac, C. Saint-Martin, M. Ruberg, C. Dravet, R. Nabbout, M. Baulac, I. Gourfinkel-An, and E. LeGuern. Parental mosaicism can cause recurrent transmission of SCN1A mutations associated with severe myoclonic epilepsy of infancy. Hum Mutat, 27(4):389, 2006. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW]



29. O. Dubourg, H. Azzedine, C. Verny, G. Durosier, N. Birouk, R. Gouider, M. Salih, A. Bouhouche, A. Thiam, D. Grid, M. Mayer, M. Ruberg, M. Tazir, A. Brice, and E. LeGuern. Autosomal-recessive forms of demyelinating Charcot-Marie-Tooth disease. Neuromolecular Med, 8(1-2):75-86, 2006. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW]



30. O. Dubourg, H. Azzedine, R. B. Yaou, J. Pouget, A. Barois, V. Meininger, D. Bouteiller, M. Ruberg, A. Brice, and E. LeGuern. The G526R glycyl-tRNA synthetase gene mutation in distal hereditary motor neuropathy type V. Neurology, 66(11):1721-6, 2006. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Age of Onset, DNA Mutational Analysis, Family, Female, France/epidemiology, Genetic Predisposition to Disease/epidemiology/genetics, Glycine-tRNA Ligase/*genetics, Heterozygote, Humans, Incidence, Jews/genetics, Muscular Atrophy, Spinal/*epidemiology/*genetics, Mutation, Risk Assessment/*methods, Risk Factors.



31. A. Janer, E. Martin, M. P. Muriel, M. Latouche, H. Fujigasaki, M. Ruberg, A. Brice, Y. Trottier, and A. Sittler. PML clastosomes prevent nuclear accumulation of mutant ataxin-7 and other polyglutamine proteins. J Cell Biol, 174(1):65-76, 2006. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Animals, COS Cells, Cadmium Chloride/pharmacology, Cell Nucleus/*metabolism, Cells, Cultured, Cercopithecus aethiops, Humans, Interferon-beta/pharmacology, Mice, Mice, Transgenic, Multiprotein Complexes/drug effects/*metabolism, Mutation, Neoplasm Proteins/drug effects/*metabolism, Nerve Tissue Proteins/genetics/*metabolism, Nuclear Proteins/drug effects/*metabolism, Peptides/genetics, Proteasome Endopeptidase Complex/drug effects/*metabolism, Protein Isoforms/drug effects/metabolism, Transcription Factors/drug effects/*metabolism, Tumor Suppressor Proteins/drug effects/*metabolism.



32. A. Lannuzel, G. U. Hoglinger, P. Champy, P. P. Michel, E. C. Hirsch, and M. Ruberg. Is atypical parkinsonism in the Caribbean caused by the consumption of Annonacae?. J Neural Transm Suppl, (70):153-7, 2006. Note: Journal ArticleAustria. [WWW] Keyword(s): Adenosine Triphosphate/metabolism, Alkaloids/isolation & purification/toxicity, Animals, Annonaceae/*adverse effects, Cells, Cultured, Fruit, Furans/isolation & purification/toxicity, Lactones/isolation & purification/toxicity, Male, Medicine, Herbal, Mesencephalon/cytology, Neostriatum/pathology, Nerve Degeneration/chemically induced/pathology, Parkinson Disease, Secondary/*chemically induced/epidemiology, Plant Extracts/adverse effects, Plant Leaves/chemistry, Rats, Rats, Inbred Lew, Substantia Nigra/pathology, West Indies/epidemiology.



33. M. Latouche, P. Fragner, E. Martin, K. H. El Hachimi, C. Zander, A. Sittler, M. Ruberg, A. Brice, and G. Stevanin. Polyglutamine and polyalanine expansions in ataxin7 result in different types of aggregation and levels of toxicity. Mol Cell Neurosci, 31(3):438-45, 2006. Note: Comparative StudyJournal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Animals, Brain/*metabolism/pathology/physiopathology, Cell Line, Cells, Cultured, Humans, Intranuclear Inclusion Bodies/genetics/*metabolism/pathology, Molecular Chaperones/genetics/metabolism, Nerve Tissue Proteins/genetics/*metabolism/toxicity, Neurofibrils/genetics/metabolism/pathology, Neurons/*metabolism/pathology, Peptides/*metabolism, Proteasome Endopeptidase Complex/genetics/metabolism, Protein Folding, Rats, Spinocerebellar Ataxias/genetics/metabolism/physiopathology.



34. P. P. Michel, M. Ruberg, and E. Hirsch. Dopaminergic neurons reduced to silence by oxidative stress: an early step in the death cascade in Parkinson's disease?. Sci STKE, 2006(332):pe19, 2006. Note: Journal ArticleUnited Statessignal transduction knowledge environment. [WWW] Keyword(s): Animals, Cell Death, Dopamine/metabolism, Mice, Mitochondria/metabolism, Neurons/*metabolism/pathology/physiology, *Oxidative Stress, Parkinson Disease/*metabolism/pathology/physiopathology, Signal Transduction.



35. M. Namekawa, I. Nelson, P. Ribai, A. Durr, E. Denis, G. Stevanin, M. Ruberg, and A. Brice. A founder effect and mutational hot spots may contribute to the most frequent mutations in the SPG3A gene. Neurogenetics, 7(2):131-2, 2006. Note: LetterResearch Support, Non-U.S. Gov'tUnited States. [WWW]



36. M. Namekawa, P. Ribai, I. Nelson, S. Forlani, F. Fellmann, C. Goizet, C. Depienne, G. Stevanin, M. Ruberg, A. Durr, and A. Brice. SPG3A is the most frequent cause of hereditary spastic paraplegia with onset before age 10 years. Neurology, 66(1):112-4, 2006. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Adolescent, Adult, Age of Onset, Aged, Brain/pathology/physiopathology, DNA Mutational Analysis, Family Health, GTP Phosphohydrolases/*genetics, Genetic Predisposition to Disease/*genetics, Genetic Screening, Humans, Male, Middle Aged, Mutation/*genetics, Peripheral Nerves/pathology/physiopathology, Phenotype, Spastic Paraplegia, Hereditary/diagnosis/*genetics/physiopathology, Wallerian Degeneration/diagnosis/genetics.



37. P. Rizk, J. Salazar, R. Raisman-Vozari, M. Marien, M. Ruberg, F. Colpaert, and T. Debeir. The alpha2-adrenoceptor antagonist dexefaroxan enhances hippocampal neurogenesis by increasing the survival and differentiation of new granule cells. Neuropsychopharmacology, 31(6):1146-57, 2006. Note: Comparative StudyJournal ArticleResearch Support, Non-U.S. Gov'tUnited Statesofficial publication of the American College of Neuropsychopharmacology. [WWW] Keyword(s): Animals, Benzopyrans/*pharmacology, Brain-Derived Neurotrophic Factor/metabolism, Bromodeoxyuridine/diagnostic use, Cell Count/methods, Cell Differentiation/*drug effects, Cell Survival/drug effects, Chromatography, High Pressure Liquid/methods, Hippocampus/*cytology, Imidazoles/*pharmacology, Immunohistochemistry/methods, In Situ Nick-End Labeling/methods, Male, Microdialysis/methods, Neural Cell Adhesion Molecule L1/metabolism, Neurons/*drug effects, Norepinephrine/metabolism, Rats, Rats, Sprague-Dawley, Receptors, Adrenergic, alpha-2/*antagonists & inhibitors, Sialic Acids/metabolism, Time Factors.



38. P. G. Unschuld, J. Dachsel, F. Darios, A. Kohlmann, E. Casademunt, K. Lehmann-Horn, M. Dichgans, M. Ruberg, A. Brice, T. Gasser, and C. B. Lucking. Parkin modulates gene expression in control and ceramide-treated PC12 cells. Mol Biol Rep, 33(1):13-32, 2006. Note: Journal ArticleResearch Support, Non-U.S. Gov'tNetherlands. [WWW]



39. N. Bouslam, A. Benomar, H. Azzedine, A. Bouhouche, M. Namekawa, S. Klebe, C. Charon, A. Durr, M. Ruberg, A. Brice, M. Yahyaoui, and G. Stevanin. Mapping of a new form of pure autosomal recessive spastic paraplegia (SPG28). Ann Neurol, 57(4):567-71, 2005. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Adult, *Chromosome Mapping, *Chromosomes, Human, Pair 14, Female, Humans, Lod Score, Male, Microsatellite Repeats, Middle Aged, Morocco, Pedigree, Polymerase Chain Reaction, Spastic Paraplegia, Hereditary/*genetics.



40. P. Champy, A. Melot, V. Guerineau Eng, C. Gleye, D. Fall, G. U. Hoglinger, M. Ruberg, A. Lannuzel, O. Laprevote, A. Laurens, and R. Hocquemiller. Quantification of acetogenins in Annona muricata linked to atypical parkinsonism in guadeloupe. Mov Disord, 20(12):1629-33, 2005. Note: Comparative StudyJournal ArticleResearch Support, Non-U.S. Gov'tUnited Statesofficial journal of the Movement Disorder Society. [WWW] Keyword(s): Annona/*chemistry/toxicity, Chromatography, High Pressure Liquid/methods, Fatty Alcohols/*adverse effects/*analysis/chemistry, Guadeloupe/epidemiology, Humans, Lactones/*adverse effects/*analysis/chemistry, Molecular Weight, Parkinsonian Disorders/*etiology, Plant Extracts/adverse effects/analysis/chemistry, Spectrometry, Mass, Matrix-Assisted Laser Desorption-Ionization/methods.



41. O. Corti, C. Hampe, F. Darios, P. Ibanez, M. Ruberg, and A. Brice. Parkinson's disease: from causes to mechanisms. C R Biol, 328(2):131-42, 2005. Note: Journal ArticleReviewFrance. [WWW] Keyword(s): Corpus Striatum/physiopathology, Dopamine/physiology, Humans, Mitochondria/pathology/physiology, Nerve Tissue Proteins/genetics, Parkinson Disease/etiology/genetics/*physiopathology, Substantia Nigra/physiopathology, Synucleins, Ubiquitin-Protein Ligases/genetics.



42. F. Darios, M. P. Muriel, M. E. Khondiker, A. Brice, and M. Ruberg. Neurotoxic calcium transfer from endoplasmic reticulum to mitochondria is regulated by cyclin-dependent kinase 5-dependent phosphorylation of tau. J Neurosci, 25(16):4159-68, 2005. Note: Comparative StudyJournal ArticleResearch Support, Non-U.S. Gov'tUnited Statesthe official journal of the Society for Neuroscience. [WWW] Keyword(s): Analysis of Variance, Animals, Blotting, Western/methods, Calcium/*metabolism, Cell Count/methods, Cell Death/drug effects, Cells, Cultured, Ceramides/pharmacology, Cloning, Molecular/methods, Cyclin-Dependent Kinase 5/*physiology, Cytochalasin D/pharmacology, Drug Interactions, Embryo, Endoplasmic Reticulum/*metabolism/ultrastructure, Enzyme Activation/drug effects, Enzyme Inhibitors/pharmacology, Female, Gene Expression Regulation/drug effects, Heterocyclic Compounds, 3-Ring/diagnostic use, Immunohistochemistry/methods, Male, Mesencephalon/cytology, Microscopy, Electron, Transmission/methods, Mitochondria/*metabolism/ultrastructure, Neurons/drug effects/physiology/ultrastructure, Nocodazole/pharmacology, Phosphorylation, Pregnancy, Purines/pharmacology, Rats, Rats, Wistar, Time Factors, Transfection/methods, Tubulin/metabolism, tau Proteins/genetics/*metabolism.



43. G. U. Hoglinger, A. Lannuzel, M. E. Khondiker, P. P. Michel, C. Duyckaerts, J. Feger, P. Champy, A. Prigent, F. Medja, A. Lombes, W. H. Oertel, M. Ruberg, and E. C. Hirsch. The mitochondrial complex I inhibitor rotenone triggers a cerebral tauopathy. J Neurochem, 95(4):930-9, 2005. Note: Comparative StudyJournal ArticleResearch Support, Non-U.S. Gov'tEngland. [WWW] Keyword(s): Amyloid beta-Protein/metabolism, Analysis of Variance, Animals, Antineoplastic Combined Chemotherapy Protocols/metabolism, Behavior, Animal, Body Weight/drug effects, Caspase 3, Caspases/metabolism, Cell Death/drug effects, Cerebral Cortex/*drug effects/pathology/physiopathology, Cytarabine/metabolism, Diagnostic Imaging/methods, Dopamine and cAMP-Regulated Phosphoprotein 32/metabolism, Doxorubicin/metabolism, Dystonia/etiology/physiopathology, Electron Transport Complex III/metabolism, Enzyme Activation/drug effects, Glial Fibrillary Acidic Protein/metabolism, Immunohistochemistry/methods, Locomotion/drug effects, Male, Microscopy, Electron, Transmission/methods, Mitochondria/drug effects/ultrastructure, Neurons/drug effects/physiology, Phosphopyruvate Hydratase/metabolism, Phosphorylation, Posture, Psychomotor Performance/drug effects, Rats, Rats, Inbred Lew, Rotenone/*adverse effects, Tauopathies/*chemically induced/pathology/physiopathology, Thiazoles/diagnostic use, Time Factors, Tyrosine/analogs & derivatives/metabolism, Tyrosine 3-Monooxygenase/metabolism, Ubiquitin/metabolism, Uncoupling Agents/*adverse effects, alpha-Synuclein/metabolism, tau Proteins/metabolism.



44. G. U. Hoglinger, P. P. Michel, P. Champy, J. Feger, E. C. Hirsch, M. Ruberg, and A. Lannuzel. Experimental evidence for a toxic etiology of tropical parkinsonism. Mov Disord, 20(1):118-9, 2005. Note: CommentComparative StudyLetterUnited Statesofficial journal of the Movement Disorder Society. [WWW] Keyword(s): Adenosine Triphosphate/metabolism, Alkaloids/toxicity, Animals, Annonaceae/chemistry/*toxicity, Brain/cytology/drug effects, Cells, Cultured, Fatty Alcohols/toxicity, Humans, Lactones/toxicity, Mitochondrial Diseases/complications/etiology, Neurons/drug effects, Pacific Islands, Parkinsonian Disorders/*etiology, Rats.



45. S. Mourlevat, T. Debeir, J. E. Ferrario, J. Delbe, D. Caruelle, O. Lejeune, C. Depienne, J. Courty, R. Raisman-Vozari, and M. Ruberg. Pleiotrophin mediates the neurotrophic effect of cyclic AMP on dopaminergic neurons: analysis of suppression-subtracted cDNA libraries and confirmation in vitro. Exp Neurol, 194(1):243-54, 2005. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Animals, Carrier Proteins/*genetics/physiology, Cells, Cultured, Cyclic AMP/*metabolism, Cytokines/*genetics/physiology, Dopamine/metabolism, Drug Resistance/genetics, Enzyme Activation/physiology, Gene Expression Profiling, Gene Expression Regulation/physiology, Gene Library, Genetic Predisposition to Disease/genetics, Membrane Glycoproteins/genetics, Nerve Degeneration/*metabolism/pathology/physiopathology, Nerve Growth Factors/*metabolism, Nerve Tissue Proteins/genetics, Neurons/*metabolism/pathology, Oligonucleotide Array Sequence Analysis, Parkinsonian Disorders/metabolism/pathology/physiopathology, Peptide Hydrolases/metabolism, Protein-Tyrosine-Phosphatase/genetics, Proteoglycans/genetics, Rats, Substantia Nigra/*metabolism/pathology/physiopathology, Syndecan-3.



46. M. Naimi, S. Tardieu, C. Depienne, M. Ruberg, A. Brice, O. Dubourg, and E. Leguern. Detection of genomic rearrangements by DHPLC: a prospective study of 90 patients with inherited peripheral neuropathies associated with 17p11.2 rearrangements. Am J Med Genet A, 136(2):136-9, 2005. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Charcot-Marie-Tooth Disease/genetics/pathology, Chromatography, High Pressure Liquid/*methods, *Chromosome Aberrations, Chromosomes, Human, Pair 17/genetics, DNA/genetics, Gene Dosage, Hereditary Motor and Sensory Neuropathies/genetics/pathology, Humans, Myelin Proteins/genetics, Paralysis/genetics/pathology, Polymerase Chain Reaction, Prospective Studies, Reproducibility of Results.



47. P. Champy, G. U. Hoglinger, J. Feger, C. Gleye, R. Hocquemiller, A. Laurens, V. Guerineau, O. Laprevote, F. Medja, A. Lombes, P. P. Michel, A. Lannuzel, E. C. Hirsch, and M. Ruberg. Annonacin, a lipophilic inhibitor of mitochondrial complex I, induces nigral and striatal neurodegeneration in rats: possible relevance for atypical parkinsonism in Guadeloupe. J Neurochem, 88(1):63-9, 2004. Note: Journal ArticleResearch Support, Non-U.S. Gov'tEngland. [WWW] Keyword(s): Adenosine Triphosphate/metabolism, Animals, Behavior, Animal/drug effects, Cell Count, Corpus Striatum/*drug effects/metabolism/pathology, Electron Transport Complex I/*antagonists & inhibitors, Furans/administration & dosage/*toxicity, Gliosis/chemically induced/pathology, Guadeloupe, Infusions, Intravenous, Lactones/administration & dosage/*toxicity, Male, Mitochondria/enzymology, Neurodegenerative Diseases/*chemically induced/enzymology/pathology, Neurons/drug effects/pathology, Parkinsonian Disorders/etiology, Plant Extracts/administration & dosage/*toxicity, Rats, Rats, Inbred Lew, Substantia Nigra/*drug effects/metabolism/pathology.



48. A. Durr, A. Camuzat, E. Colin, C. Tallaksen, D. Hannequin, P. Coutinho, B. Fontaine, A. Rossi, R. Gil, C. Rousselle, M. Ruberg, G. Stevanin, and A. Brice. Atlastin1 mutations are frequent in young-onset autosomal dominant spastic paraplegia. Arch Neurol, 61(12):1867-72, 2004. Note: Comparative StudyJournal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Adolescent, Adult, Age of Onset, Aged, Aged, 80 and over, Child, Child, Preschool, Female, GTP Phosphohydrolases/*genetics, Humans, Male, Middle Aged, *Mutation, Pedigree, Spastic Paraplegia, Hereditary/*genetics.



49. J. E. Ferrario, I. R. Taravini, S. Mourlevat, A. Stefano, M. A. Delfino, R. Raisman-Vozari, M. G. Murer, M. Ruberg, and O. Gershanik. Differential gene expression induced by chronic levodopa treatment in the striatum of rats with lesions of the nigrostriatal system. J Neurochem, 90(6):1348-58, 2004. Note: Comparative StudyJournal ArticleResearch Support, Non-U.S. Gov'tEngland. [WWW] Keyword(s): Animals, Antiparkinson Agents/*pharmacology, Behavior, Animal, Brain Diseases/chemically induced/*metabolism, Calmodulin/genetics/metabolism, Carrier Proteins/genetics/metabolism, Cell Count/methods, Corpus Striatum/injuries/*metabolism, Cytokines/genetics/metabolism, Dopamine Plasma Membrane Transport Proteins, Female, Functional Laterality/physiology, Gene Expression/*drug effects, Gene Library, Immunohistochemistry/methods, In Situ Hybridization/methods, Levodopa/*pharmacology, Membrane Glycoproteins/metabolism, Membrane Transport Proteins/metabolism, Myelin Basic Proteins/genetics/metabolism, Nerve Tissue Proteins/metabolism, Oligonucleotide Array Sequence Analysis/methods, Oxidopamine, Radioimmunoassay/methods, Rats, Rats, Wistar, Substantia Nigra/*metabolism.



50. I. Gourfinkel-An, S. Baulac, R. Nabbout, M. Ruberg, M. Baulac, A. Brice, and E. LeGuern. Monogenic idiopathic epilepsies. Lancet Neurol, 3(4):209-18, 2004. Note: Journal ArticleResearch Support, Non-U.S. Gov'tReviewEngland. [WWW] Keyword(s): Animals, Anticonvulsants/therapeutic use, Epilepsy/classification/complications/drug therapy/*genetics, Epilepsy, Benign Neonatal/genetics, Epilepsy, Generalized/genetics, Epilepsy, Temporal Lobe/genetics, Humans, Ion Channels/chemistry/drug effects/*genetics, *Mutation, Receptors, Neurotransmitter/chemistry/drug effects/*genetics, Seizures, Febrile/genetics.



51. B. Salthun-Lassalle, E. C. Hirsch, J. Wolfart, M. Ruberg, and P. P. Michel. Rescue of mesencephalic dopaminergic neurons in culture by low-level stimulation of voltage-gated sodium channels. J Neurosci, 24(26):5922-30, 2004. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited Statesthe official journal of the Society for Neuroscience. [WWW] Keyword(s): Animals, Apamin/pharmacology, Apoptosis/drug effects, Aspirin/pharmacology, Biological Transport, Active/drug effects, Calcium Channel Agonists/pharmacology, Calcium Channel Blockers/pharmacology, Calcium Channels, T-Type/drug effects, Cells, Cultured/cytology/drug effects/metabolism, Dopamine/*metabolism, Glial Cell Line-Derived Neurotrophic Factor, Ion Channel Gating, Ion Transport/drug effects, Mesencephalon/*cytology, Na(+)-K(+)-Exchanging ATPase/antagonists & inhibitors/physiology, Nerve Growth Factors/pharmacology, Nerve Tissue Proteins/*drug effects/physiology, Neurons/cytology/*drug effects/metabolism, Neuroprotective Agents/*pharmacology, Nifedipine/pharmacology, Ouabain/pharmacology, Potassium Channel Blockers/pharmacology, Potassium Channels, Calcium-Activated/antagonists & inhibitors, Rats, Rats, Wistar, Scorpion Venoms/pharmacology, Serotonin/metabolism, Sodium/pharmacology/*physiology, Sodium Channel Blockers/*pharmacology, Sodium Channels/*drug effects/physiology, Tetrodotoxin/pharmacology, Veratridine/*pharmacology, gamma-Aminobutyric Acid/metabolism.



52. G. Stevanin, V. Hahn, E. Lohmann, N. Bouslam, M. Gouttard, C. Soumphonphakdy, M. L. Welter, E. Ollagnon-Roman, A. Lemainque, M. Ruberg, A. Brice, and A. Durr. Mutation in the catalytic domain of protein kinase C gamma and extension of the phenotype associated with spinocerebellar ataxia type 14. Arch Neurol, 61(8):1242-8, 2004. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Adult, Aged, Amino Acid Sequence, Base Sequence, Brain/pathology, Catalytic Domain/*genetics, Chromosomes, Human, Pair 19/enzymology/genetics, Female, Humans, Linkage (Genetics)/genetics, Male, Middle Aged, Molecular Sequence Data, *Mutation, Missense, Pedigree, *Phenotype, Protein Kinase C/*genetics, Spinocerebellar Ataxias/*enzymology/*genetics/pathology.



53. H. Azzedine, M. Ruberg, D. Ente, C. Gilardeau, S. Perie, B. Wechsler, A. Brice, E. LeGuern, and O. Dubourg. Variability of disease progression in a family with autosomal recessive CMT associated with a S194X and new R310Q mutation in the GDAP1 gene. Neuromuscul Disord, 13(4):341-6, 2003. Note: Case ReportsJournal ArticleResearch Support, Non-U.S. Gov'tEnglandNmd. [WWW] Keyword(s): Adult, Arginine/genetics, Charcot-Marie-Tooth Disease/*genetics, DNA Mutational Analysis, Disease Progression, Electrophysiology, Family, Female, Genes, Recessive, Glutamine/genetics, Humans, Male, *Mutation, Nerve Tissue Proteins/*genetics, Pedigree, Phenotype, Serine/genetics.



54. O. Corti, C. Hampe, H. Koutnikova, F. Darios, S. Jacquier, A. Prigent, J. C. Robinson, L. Pradier, M. Ruberg, M. Mirande, E. Hirsch, T. Rooney, A. Fournier, and A. Brice. The p38 subunit of the aminoacyl-tRNA synthetase complex is a Parkin substrate: linking protein biosynthesis and neurodegeneration. Hum Mol Genet, 12(12):1427-37, 2003. Note: Journal ArticleResearch Support, Non-U.S. Gov'tEngland. [WWW] Keyword(s): Adult, Amino Acyl-tRNA Synthetases/*metabolism, Animals, COS Cells, Carrier Proteins/*metabolism, Cell Death/*drug effects, Cercopithecus aethiops, Humans, Inclusion Bodies, Lewy Bodies/metabolism/pathology, Mesencephalon/*metabolism, Mutation, Nerve Degeneration/metabolism/pathology, Neuroblastoma/metabolism/pathology, Parkinson Disease/*genetics/physiopathology, Saccharomyces cerevisiae, Two-Hybrid System Techniques, Ubiquitin/metabolism, Ubiquitin-Protein Ligases/genetics/*metabolism, p38 Mitogen-Activated Protein Kinases.



55. F. Darios, O. Corti, C. B. Lucking, C. Hampe, M. P. Muriel, N. Abbas, W. J. Gu, E. C. Hirsch, T. Rooney, M. Ruberg, and A. Brice. Parkin prevents mitochondrial swelling and cytochrome c release in mitochondria-dependent cell death. Hum Mol Genet, 12(5):517-26, 2003. Note: Journal ArticleResearch Support, Non-U.S. Gov'tEngland. [WWW] Keyword(s): Animals, Caspase 3, Caspases/metabolism, Cell Death/physiology, Ceramides/metabolism, Cytochrome c Group/*metabolism, Humans, Ligases/*metabolism, Mitochondria/*metabolism, Neurons/metabolism, Rats, Ubiquitin-Protein Ligases.



56. F. Darios, N. Lambeng, J. D. Troadec, P. P. Michel, and M. Ruberg. Ceramide increases mitochondrial free calcium levels via caspase 8 and Bid: role in initiation of cell death. J Neurochem, 84(4):643-54, 2003. Note: Journal ArticleResearch Support, Non-U.S. Gov'tEngland. [WWW] Keyword(s): Animals, Apoptosis/drug effects/physiology, BH3 Interacting Domain Death Agonist Protein, Calcium/*metabolism, Carrier Proteins/*metabolism, Caspase 8, Caspase 9, Caspases/*metabolism, Cell Differentiation/drug effects, Ceramides/*pharmacology, Cytochrome c Group/metabolism, Endoplasmic Reticulum/metabolism, Mitochondria/drug effects/*metabolism, Nerve Growth Factor/pharmacology, Neurons/drug effects/metabolism, PC12 Cells, Rats, Reactive Oxygen Species/metabolism, Sphingosine/*analogs & derivatives/pharmacology.



57. W. J. Gu, O. Corti, F. Araujo, C. Hampe, S. Jacquier, C. B. Lucking, N. Abbas, C. Duyckaerts, T. Rooney, L. Pradier, M. Ruberg, and A. Brice. The C289G and C418R missense mutations cause rapid sequestration of human Parkin into insoluble aggregates. Neurobiol Dis, 14(3):357-64, 2003. Note: 1r01ns41723-01a1/ns/nindsJournal ArticleResearch Support, Non-U.S. Gov'tResearch Support, U.S. Gov't, P.H.S.United States. [WWW] Keyword(s): Animals, COS Cells, Cysteine Endopeptidases/metabolism, Humans, Inclusion Bodies/*genetics/metabolism, Macromolecular Substances, Microtubules/metabolism, Multienzyme Complexes/metabolism, Mutation, Missense/*genetics, Neurons/*metabolism/pathology, Parkinson Disease/*genetics/metabolism/physiopathology, Proteasome Endopeptidase Complex, Protein Folding, Protein Structure, Tertiary/genetics, Protein Transport/physiology, Solubility, Ubiquitin-Protein Ligases/genetics/*metabolism, Ubiquitins/metabolism.



58. E. C. Hirsch, G. Hoglinger, E. Rousselet, T. Breidert, K. Parain, J. Feger, M. Ruberg, A. Prigent, C. Cohen-Salmon, and J. M. Launay. Animal models of Parkinson's disease in rodents induced by toxins: an update. J Neural Transm Suppl, (65):89-100, 2003. Note: Journal ArticleReviewAustria. [WWW] Keyword(s): 1-Methyl-4-phenyl-1, 2, 3, 6-tetrahydropyridine, Adrenergic Agents, Animals, Basal Ganglia/drug effects/metabolism, Brain Stem/drug effects/metabolism, *Disease Models, Animal, Dopamine/metabolism, Dopamine Agents, Nerve Degeneration/*chemically induced, Neurons/*drug effects, Oxidopamine, *Parkinson Disease, *Rodentia, Rotenone, Uncoupling Agents.



59. G. U. Hoglinger, G. Carrard, P. P. Michel, F. Medja, A. Lombes, M. Ruberg, B. Friguet, and E. C. Hirsch. Dysfunction of mitochondrial complex I and the proteasome: interactions between two biochemical deficits in a cellular model of Parkinson's disease. J Neurochem, 86(5):1297-307, 2003. Note: Journal ArticleResearch Support, Non-U.S. Gov'tEngland. [WWW] Keyword(s): 1-Methyl-4-phenylpyridinium/toxicity, Adenosine Triphosphate/metabolism, Animals, Cell Death, Cells, Cultured, Cysteine Endopeptidases/*metabolism, Dose-Response Relationship, Drug, Electron Transport Complex I, Enzyme Inhibitors/toxicity, Mesencephalon/cytology/embryology, Multienzyme Complexes/antagonists & inhibitors/*metabolism, NADH, NADPH Oxidoreductases/antagonists & inhibitors/*metabolism, Neurons/drug effects/*metabolism/pathology, Neurotoxins/toxicity, Oxidation-Reduction/drug effects, Parkinsonian Disorders/*metabolism/pathology, Proteasome Endopeptidase Complex, Rats, Rats, Wistar, Reactive Oxygen Species/metabolism, Rotenone/toxicity, Uncoupling Agents/toxicity.



60. G. U. Hoglinger, J. Feger, A. Prigent, P. P. Michel, K. Parain, P. Champy, M. Ruberg, W. H. Oertel, and E. C. Hirsch. Chronic systemic complex I inhibition induces a hypokinetic multisystem degeneration in rats. J Neurochem, 84(3):491-502, 2003. Note: Journal ArticleResearch Support, Non-U.S. Gov'tEngland. [WWW] Keyword(s): Animals, Behavior, Animal/drug effects, Cell Count, Choline O-Acetyltransferase/biosynthesis, Chronic Disease, Corpus Striatum/drug effects/pathology, Disease Models, Animal, Dopamine and cAMP-Regulated Phosphoprotein 32, Drug Administration Schedule, Electron Transport Complex I, Enzyme Inhibitors/administration & dosage/*toxicity, Inclusion Bodies/pathology, Infusions, Intravenous, Locus Coeruleus/drug effects/pathology, Male, Mesencephalon/drug effects/pathology, Motor Activity/drug effects, Movement Disorders/complications/etiology/*pathology, NADH, NADPH Oxidoreductases/*antagonists & inhibitors, *Nerve Tissue Proteins, Neurodegenerative Diseases/chemically induced/complications/*pathology, Neuroglia/pathology, Neurons/drug effects/metabolism/pathology, Pesticides/toxicity, Phosphoproteins/biosynthesis, Rats, Rats, Inbred Lew, Rotenone/administration & dosage/*toxicity, Serotonin/biosynthesis, Substantia Nigra/drug effects/pathology, Time, Tyrosine 3-Monooxygenase/biosynthesis.



61. A. Lannuzel, P. P. Michel, G. U. Hoglinger, P. Champy, A. Jousset, F. Medja, A. Lombes, F. Darios, C. Gleye, A. Laurens, R. Hocquemiller, E. C. Hirsch, and M. Ruberg. The mitochondrial complex I inhibitor annonacin is toxic to mesencephalic dopaminergic neurons by impairment of energy metabolism. Neuroscience, 121(2):287-96, 2003. Note: Comparative StudyJournal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): 1-Methyl-4-phenylpyridinium/toxicity, Acetylcysteine/pharmacology, Adenosine Triphosphate/analysis, Animals, Antioxidants/pharmacology, Benzodiazepines/pharmacology, Cell Survival, Cells, Cultured, Chromans/pharmacology, Deoxyglucose/metabolism, Dizocilpine Maleate/pharmacology, Dopamine/*metabolism, Dose-Response Relationship, Drug, Drug Interactions, Embryo, Energy Metabolism/*drug effects/physiology, Excitatory Amino Acid Antagonists, Female, Furans/chemistry/*toxicity, Glucose/pharmacology, Herbicides/toxicity, Hexoses/pharmacology, Insecticides/toxicity, Intracellular Space/metabolism, Lactones/chemistry/*toxicity, Male, Mannose/pharmacology, Mesencephalon/cytology/*drug effects/physiology, Microtubule-Associated Proteins/metabolism, Mitochondria/*drug effects, Neurons/*drug effects/physiology, Neurotoxins/chemistry/*toxicity, Plant Extracts/chemistry/toxicity, Pregnancy, Rats, Rats, Wistar, Reactive Oxygen Species, Rotenone/toxicity, Tritium/metabolism, Tyrosine 3-Monooxygenase/metabolism.



62. E. Lohmann, M. Periquet, V. Bonifati, N. W. Wood, G. De Michele, A. M. Bonnet, V. Fraix, E. Broussolle, M. W. Horstink, M. Vidailhet, P. Verpillat, T. Gasser, D. Nicholl, H. Teive, S. Raskin, O. Rascol, A. Destee, M. Ruberg, F. Gasparini, G. Meco, Y. Agid, A. Durr, and A. Brice. How much phenotypic variation can be attributed to parkin genotype?. Ann Neurol, 54(2):176-85, 2003. Note: French Parkinson's Disease Genetics Study GroupEuropean Consortium on Genetic Susceptibility in Parkinson's DiseaseNs41723-01a1/ns/nindsJournal ArticleResearch Support, Non-U.S. Gov'tResearch Support, U.S. Gov't, P.H.S.United States. [WWW] Keyword(s): Adolescent, Adult, Age of Onset, Aged, Aged, 80 and over, Antiparkinson Agents/therapeutic use, Disease Progression, Exons/genetics, Female, Genotype, Heterozygote, Humans, Levodopa/therapeutic use, Male, Middle Aged, Mutation/genetics, Mutation, Missense/genetics, Parkinson Disease/drug therapy/*genetics/physiopathology, Phenotype, Ubiquitin-Protein Ligases/*genetics, Variation (Genetics)/*genetics.



63. S. Mourlevat, J. D. Troadec, M. Ruberg, and P. P. Michel. Prevention of dopaminergic neuronal death by cyclic AMP in mixed neuronal/glial mesencephalic cultures requires the repression of presumptive astrocytes. Mol Pharmacol, 64(3):578-86, 2003. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Animals, Astrocytes/*drug effects/physiology, Cell Death/drug effects/physiology, Coculture Techniques, Cyclic AMP/*pharmacology, Dopamine/*physiology, Female, Mesencephalon/cytology/*drug effects/physiology, Neuroglia/drug effects/physiology, Neurons/*drug effects/physiology, Rats, Rats, Wistar.



64. N. Ravise, O. Dubourg, S. Tardieu, F. Aurias, M. Mercadiel, P. Coullin, M. Ruberg, M. Catala, S. Lesourd, A. Brice, and E. LeGuern. Rapid detection of 17p11.2 rearrangements by FISH without cell culture (direct FISH, DFISH): a prospective study of 130 patients with inherited peripheral neuropathies. Am J Med Genet A, 118(1):43-8, 2003. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Charcot-Marie-Tooth Disease/diagnosis/genetics, *Chromosome Aberrations, *Chromosomes, Human, Pair 17, Humans, In Situ Hybridization, Fluorescence, Paralysis/genetics, Peripheral Nervous System Diseases/diagnosis/genetics.



65. C. M. Tallaksen, E. Guichart-Gomez, P. Verpillat, V. Hahn-Barma, M. Ruberg, B. Fontaine, A. Brice, B. Dubois, and A. Durr. Subtle cognitive impairment but no dementia in patients with spastin mutations. Arch Neurol, 60(8):1113-8, 2003. Note: Comparative StudyJournal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Adenosine Triphosphatases/*genetics, Adolescent, Adult, Age of Onset, Child, Child, Preschool, *Cognition, DNA Mutational Analysis, *Dementia/genetics, Disease Progression, Female, *Heterozygote, Humans, Infant, Male, Middle Aged, Mutation, Neuropsychological Tests, Spastic Paraplegia, Hereditary/genetics/*physiopathology.

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