1. 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.



2. A. C. Bruni, J. Takahashi-Fujigasaki, F. Maltecca, J. F. Foncin, A. Servadio, G. Casari, P. D'Adamo, R. Maletta, S. A. Curcio, G. De Michele, A. Filla, K. H. El Hachimi, and C. Duyckaerts. Behavioral disorder, dementia, ataxia, and rigidity in a large family with TATA box-binding protein mutation. Arch Neurol, 61(8):1314-20, 2004. Note: Case ReportsJournal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Adult, Aged, Behavioral Symptoms/genetics/pathology/psychology, Brain/pathology, Dementia/*genetics/pathology/psychology, Female, Humans, Male, Middle Aged, Muscle Rigidity/*genetics/pathology/psychology, *Mutation, Pedigree, Spinocerebellar Ataxias/*genetics/pathology/psychology, TATA-Box Binding Protein/*genetics.



3. D. Langui, N. Girardot, K. H. El Hachimi, B. Allinquant, V. Blanchard, L. Pradier, and C. Duyckaerts. Subcellular topography of neuronal Abeta peptide in APPxPS1 transgenic mice. Am J Pathol, 165(5):1465-77, 2004. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Amyloid beta-Protein/genetics/*physiology, Amyloid beta-Protein Precursor/genetics, Animals, Biotinylation, Blotting, Western, Brain/pathology, Cathepsin D/physiology, Epitopes/chemistry, Genotype, Golgi Apparatus/metabolism, Immunohistochemistry, Lysosomes/metabolism, Membrane Proteins/genetics/*physiology, Mice, Mice, Inbred C57BL, Mice, Transgenic, Microscopy, Electron, Microscopy, Fluorescence, Microscopy, Immunoelectron, Models, Biological, Neurons/*metabolism, Peptides/chemistry, Presenilin-1, Protein Structure, Tertiary.



4. A. Mouatt-Prigent, M. P. Muriel, W. J. Gu, K. H. El Hachimi, C. B. Lucking, A. Brice, and E. C. Hirsch. Ultrastructural localization of parkin in the rat brainstem, thalamus and basal ganglia. J Neural Transm, 111(10-11):1209-18, 2004. Note: Journal ArticleResearch Support, Non-U.S. Gov'tAustria1996). [WWW] Keyword(s): Animals, Anti-Bacterial Agents/pharmacology, Basal Ganglia/*metabolism/*ultrastructure, Brain Stem/*metabolism/*ultrastructure, Dendrites/drug effects/metabolism/ultrastructure, Doxycycline/pharmacology, Endoplasmic Reticulum/drug effects/metabolism/ultrastructure, Immunohistochemistry, Male, Mitochondria/drug effects/metabolism/ultrastructure, Neuroglia/drug effects/metabolism/ultrastructure, Neurons/drug effects/metabolism/ultrastructure, PC12 Cells, Rats, Rats, Wistar, Synapses/drug effects/metabolism/ultrastructure, Thalamus/*metabolism/*ultrastructure, Ubiquitin-Protein Ligases/*biosynthesis/*genetics.



5. D. Seilhean, J. Takahashi, K. H. El Hachimi, H. Fujigasaki, A. S. Lebre, V. Biancalana, A. Durr, F. Salachas, J. Hogenhuis, H. de The, J. J. Hauw, V. Meininger, A. Brice, and C. Duyckaerts. Amyotrophic lateral sclerosis with neuronal intranuclear protein inclusions. Acta Neuropathol (Berl), 108(1):81-7, 2004. Note: Case ReportsJournal ArticleResearch Support, Non-U.S. Gov'tGermany. [WWW] Keyword(s): Amyotrophic Lateral Sclerosis/*metabolism, Cysteine Endopeptidases/metabolism, Female, Hippocampus/*pathology, Humans, Immunohistochemistry/methods, Intranuclear Inclusion Bodies/*metabolism/ultrastructure, Microscopy, Electron/methods, Middle Aged, Multienzyme Complexes/metabolism, Neoplasm Proteins/metabolism, Nerve Tissue Proteins/metabolism, Neurons/*metabolism/ultrastructure, Nuclear Proteins/metabolism, Postmortem Changes, Proteasome Endopeptidase Complex, Repressor Proteins, Staining and Labeling, Transcription Factors/metabolism, Tumor Suppressor Proteins, Ubiquitin/metabolism.



6. J. Takahashi, H. Fujigasaki, K. Iwabuchi, A. C. Bruni, T. Uchihara, K. H. El Hachimi, G. Stevanin, A. Durr, A. S. Lebre, Y. Trottier, H. de The, J. Tanaka, J. J. Hauw, C. Duyckaerts, and A. Brice. PML nuclear bodies and neuronal intranuclear inclusion in polyglutamine diseases. Neurobiol Dis, 13(3):230-7, 2003. Note: Journal ArticleResearch Support, Non-U.S. Gov'tUnited States. [WWW] Keyword(s): Adolescent, Adult, Age of Onset, Aged, Cell Nucleus/*pathology, Central Nervous System Diseases/metabolism/*pathology, Child, Child, Preschool, Female, Humans, Immunohistochemistry, Inclusion Bodies/*metabolism/pathology, Male, Middle Aged, Mutation, Neoplasm Proteins/*metabolism, Neurons/metabolism/*pathology, *Nuclear Proteins, Peptides/genetics/*metabolism, Transcription Factors/*metabolism, Tumor Suppressor Proteins.

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