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Neurobiology of Disease, ISSN 0969-9961, 2009, Volume 37, Issue 2, pp. 284 - 293
Abstract Spinocerebellar ataxia type 3 (SCA3), or Machado–Joseph disease (MJD), is caused by the expansion of a polyglutamine repeat in the ataxin-3 protein.... 
Neurology | Polyglutamine | SCA3 | CAG repeat instability | MJD | Spinocerebellar ataxia type 3 | Intranuclear inclusion bodies | Late onset | Machado–Joseph disease | Transgenic mouse model | Machado-Joseph disease | EXPANDED POLYGLUTAMINE | AMPA NEUROTOXICITY | NEURONAL LOSS | NEUROSCIENCES | CEREBELLAR-ATAXIA | MACHADO-JOSEPH-DISEASE | IN-VIVO | INTERGENERATIONAL INSTABILITY | INTRANUCLEAR INCLUSIONS | DENTATORUBRAL-PALLIDOLUYSIAN ATROPHY | HUNTINGTONS-DISEASE | Ataxin-3 | Gene Expression - genetics | Machado-Joseph Disease - metabolism | Trinucleotide Repeats - genetics | Brain - metabolism | Nuclear Proteins - genetics | Disease Models, Animal | Genetic Predisposition to Disease - genetics | Brain - physiopathology | Intranuclear Inclusion Bodies - genetics | Rats | Mice, Transgenic | Sex Characteristics | Transcription Factors - genetics | Mutation - genetics | Disease Progression | Intranuclear Inclusion Bodies - pathology | Chromosomal Instability - genetics | Movement Disorders - metabolism | Animals | Movement Disorders - physiopathology | Age of Onset | Brain - pathology | Machado-Joseph Disease - physiopathology | Mice | Movement Disorders - genetics | Intranuclear Inclusion Bodies - metabolism | Machado-Joseph Disease - genetics | Disease transmission | Neurons | Analysis | Spinocerebellar ataxia | Medical genetics | Genetic aspects | Models | Genetic engineering
Journal Article
Journal Article
Journal Article
Journal Article
Actas Espanolas de Psiquiatria, ISSN 1139-9287, 07/2011, Volume 39, Issue 4, pp. 260 - 262
Hallervorden-Spatz disease is a rare neurological disorder characterized by pyramidal and extrapyramidal manifestations, dysarthria and dementia. Its onset is... 
Basal ganglia | Hallervorden-spatz | Psicosis | Late onset | PSYCHIATRY | DISORDER | late onset | HUNTINGTONS-DISEASE | NEUROSCIENCES | Hallervorden-Spatz | PARKINSONS-DISEASE
Journal Article
Journal Article
Journal Article
Geriatrics & Gerontology International, ISSN 1444-1586, 03/2007, Volume 7, Issue 1, pp. 80 - 82
We examined five patients with late‐onset Huntington’s disease (HD), who developed chorea as an initial symptom at age 60 or later. The mean disease duration... 
genetic counseling | late‐onset | chorea | Huntington’s disease | CAG trinucleotide repeat
Journal Article