X
Search Filters
Format Format
Format Format
X
Sort by Item Count (A-Z)
Filter by Count
Journal Article (1843) 1843
Dissertation (14) 14
Book Chapter (13) 13
Magazine Article (2) 2
Conference Proceeding (1) 1
Newspaper Article (1) 1
Web Resource (1) 1
more...
Subjects Subjects
Subjects Subjects
X
Sort by Item Count (A-Z)
Filter by Count
humans (1390) 1390
machado-joseph disease (792) 792
machado-joseph-disease (771) 771
male (746) 746
female (699) 699
neurosciences (656) 656
clinical neurology (638) 638
adult (592) 592
middle aged (559) 559
machado-joseph disease - genetics (499) 499
spinocerebellar ataxia (450) 450
animals (372) 372
ataxin-3 (370) 370
ataxia (345) 345
nerve tissue proteins - genetics (317) 317
aged (308) 308
genetics & heredity (291) 291
mutation (261) 261
gene (260) 260
trinucleotide repeats (248) 248
huntingtons-disease (246) 246
biochemistry & molecular biology (242) 242
cag repeat (235) 235
neurology (218) 218
expansion (212) 212
machado-joseph disease - pathology (202) 202
polyglutamine (195) 195
dominant cerebellar-ataxia (193) 193
phenotype (192) 192
nuclear proteins - genetics (191) 191
congenital, hereditary, and neonatal diseases and abnormalities (189) 189
mice (183) 183
trinucleotide repeat (178) 178
neurodegenerative diseases (176) 176
neurodegeneration (175) 175
nerve tissue proteins - metabolism (174) 174
spinocerebellar ataxia type 3 (173) 173
spinocerebellar ataxias - genetics (171) 171
pedigree (167) 167
degeneration (165) 165
machado-joseph disease - metabolism (163) 163
adolescent (162) 162
nervous system diseases (161) 161
machado-joseph disease - physiopathology (160) 160
dentatorubral-pallidoluysian atrophy (158) 158
repressor proteins - genetics (158) 158
age of onset (153) 153
clinical-features (152) 152
disease (152) 152
proteins (151) 151
cerebellar ataxia (146) 146
atrophy (139) 139
alleles (135) 135
analysis (135) 135
cerebellum (135) 135
repressor proteins (132) 132
spinocerebellar degenerations - genetics (130) 130
trinucleotide repeat expansion (125) 125
magnetic resonance imaging (122) 122
machado-joseph disease - diagnosis (121) 121
intranuclear inclusions (117) 117
sca3 (117) 117
features (112) 112
nuclear proteins (112) 112
nuclear proteins - metabolism (112) 112
disease models, animal (110) 110
peptides - metabolism (110) 110
families (109) 109
peptides - genetics (109) 109
genotype (108) 108
protein (107) 107
brain - pathology (106) 106
genetics (106) 106
cell biology (105) 105
machado-joseph disease - complications (102) 102
brain (98) 98
mice, transgenic (98) 98
machado–joseph disease (97) 97
molecular sequence data (97) 97
repressor proteins - metabolism (97) 97
research (97) 97
ataxin (95) 95
trinucleotide repeat diseases (95) 95
transgenic mice (94) 94
polyglutamine diseases (93) 93
trinucleotide repeats - genetics (92) 92
child (91) 91
genetic aspects (91) 91
biomedicine (87) 87
expanded polyglutamine (86) 86
spinocerebellar ataxias (86) 86
young adult (86) 86
cerebellar-ataxia (85) 85
trinucleotide repeat expansion - genetics (85) 85
base sequence (83) 83
neuronal intranuclear inclusions (83) 83
pathology (83) 83
locus (80) 80
gene expression (78) 78
psychiatry (76) 76
more...
Language Language
Language Language
X
Sort by Item Count (A-Z)
Filter by Count
English (1801) 1801
Japanese (47) 47
Portuguese (44) 44
Chinese (17) 17
Spanish (14) 14
German (7) 7
French (3) 3
Russian (2) 2
Czech (1) 1
Hungarian (1) 1
Norwegian (1) 1
Polish (1) 1
more...
Publication Date Publication Date
Click on a bar to filter by decade
Slide to change publication date range


Annals of neurology, ISSN 0364-5134, 2018, Volume 84, Issue 1, pp. 64 - 77
Objective Spinocerebellar ataxia type 3 (SCA3), also known as Machado–Joseph disease, is the most common dominantly inherited ataxia. Despite advances in... 
PURKINJE NEURONS | ANDROGEN RECEPTOR | SPINAL MUSCULAR-ATROPHY | MACHADO-JOSEPH-DISEASE | MOUSE MODEL | POLYGLUTAMINE DISEASES | AUTOPHAGY | DYSFUNCTION | ANTISENSE OLIGONUCLEOTIDES | NEUROSCIENCES | NEURODEGENERATIVE DISEASES | CLINICAL NEUROLOGY | Action Potentials - genetics | Age Factors | Apoptosis - drug effects | Motor Activity - drug effects | Apoptosis - genetics | Male | Brain - metabolism | Oligonucleotides, Antisense - therapeutic use | Exploratory Behavior - drug effects | Ataxin-3 - chemistry | Female | Microfilament Proteins - metabolism | Purkinje Cells - drug effects | Action Potentials - drug effects | Gliosis - etiology | Disease Models, Animal | Machado-Joseph Disease - pathology | Calcium-Binding Proteins - metabolism | Gene Expression Regulation - genetics | Mice, Transgenic | Mutation - genetics | Brain - drug effects | Gene Expression Regulation - drug effects | Machado-Joseph Disease - drug therapy | Motor Activity - genetics | Animals | Machado-Joseph Disease - physiopathology | Gliosis - drug therapy | Mice | Purkinje Cells - pathology | RNA-Binding Proteins - metabolism | Ataxin-3 - genetics | Machado-Joseph Disease - genetics | Medical research | Therapy | Afterhyperpolarization | Medical treatment | Transgenic mice | Clinical trials | Firing rate | Trinucleotide repeats | Motors | Antisense oligonucleotides | Polyglutamine diseases | Motor task performance | Hereditary diseases | Machado-Joseph disease | Ataxia | Biocompatibility | In vivo methods and tests | Trinucleotide repeat diseases
Journal Article
Brain (London, England : 1878), ISSN 0006-8950, 2012, Volume 135, Issue 8, pp. 2428 - 2439
Journal Article
The Journal of neuroscience, ISSN 1529-2401, 2007, Volume 27, Issue 28, pp. 7418 - 7428
Spinocerebellar ataxia type 3 (SCA3) is an autosomal dominantly inherited neurodegenerative disorder caused by the expansion of a CAG repeat in the MJD1 gene... 
Spinocerebellar ataxia type 3 (SCA3) | Mouse model | Nuclear localization | Polyglutamine diseases | Neurodegenerative diseases | Machado-Joseph disease (MJD) | mouse model | spinocerebellar ataxia type 3 ( SCA3) | EXPANDED CAG REPEAT | MUTANT HUNTINGTIN | NEUROSCIENCES | CELL-DEATH | NEURONAL INTRANUCLEAR INCLUSIONS | neurodegenerative diseases | MACHADO-JOSEPH-DISEASE | nuclear localization | FUNCTIONAL-ANALYSIS | polyglutamine diseases | BODY FORMATION | HUNTINGTONS-DISEASE | TRANSGENIC MICE | Trinucleotide Repeats | Ataxin-3 | Machado-Joseph Disease - metabolism | Peptides - genetics | Ubiquitin - metabolism | Machado-Joseph Disease - mortality | Motor Activity | Tissue Distribution | Machado-Joseph Disease - complications | Cell Nucleus - metabolism | Inclusion Bodies - metabolism | Nuclear Proteins - genetics | Mental Disorders - etiology | Repressor Proteins - metabolism | Exploratory Behavior | Back - abnormalities | Repressor Proteins - genetics | Mice, Transgenic | Nuclear Proteins - metabolism | Machado-Joseph Disease - psychology | Nerve Tissue Proteins - genetics | Immunohistochemistry - methods | Nerve Tissue Proteins - metabolism | Phenotype | Animals | Staining and Labeling | Mice | Tremor - etiology | Nerve Degeneration - etiology | spinocerebellar ataxia type 3 (SCA3) | Machado–Joseph disease (MJD)
Journal Article
The Journal of neuroscience, ISSN 1529-2401, 2008, Volume 28, Issue 48, pp. 12713 - 12724
Spinocerebellar ataxia type 3 (SCA3), also known as Machado-Joseph disease (MJD), is an autosomal-dominant neurodegenerative disorder caused by a polyglutamine... 
Calcium signaling | Machado-Joseph disease | Ataxin-3 | SCA3 | Neurodegeneration | Spinocerebellar ataxia type 3 | Transgenic mouse | Stereology | Dantrolene | MJD1 | CEREBELLAR | ataxin-3 | PROTEIN | spinocerebellar ataxia type 3 | calcium signaling | neurodegeneration | stereology | FUNCTIONAL-CHARACTERIZATION | transgenic mouse | NEUROSCIENCES | IN-VITRO | MACHADO-JOSEPH-DISEASE | dantrolene | ENDOPLASMIC-RETICULUM | THERAPEUTIC-USE | GENE-PRODUCT | TRANSGENIC MICE | Substantia Nigra - physiopathology | Recovery of Function - drug effects | Substantia Nigra - pathology | Machado-Joseph Disease - metabolism | Humans | Nerve Degeneration - physiopathology | Brain - metabolism | Inositol 1,4,5-Trisphosphate Receptors - metabolism | Nerve Degeneration - prevention & control | Muscle Relaxants, Central - pharmacology | Recovery of Function - physiology | Adult | Nuclear Proteins - genetics | Disease Models, Animal | Genetic Predisposition to Disease - genetics | Pons - physiopathology | Pons - pathology | Animals, Genetically Modified | Brain - physiopathology | Mice, Inbred C57BL | Cells, Cultured | Pons - drug effects | Rats | Dantrolene - therapeutic use | Dantrolene - pharmacology | Transcription Factors - genetics | Mutation - genetics | Brain - drug effects | Machado-Joseph Disease - drug therapy | Substantia Nigra - drug effects | Animals | Calcium Signaling - drug effects | Machado-Joseph Disease - physiopathology | Mice | Calcium Signaling - genetics | Muscle Relaxants, Central - therapeutic use | Nerve Degeneration - drug therapy | Machado–Joseph disease
Journal Article
Journal Article
PloS one, ISSN 1932-6203, 2013, Volume 8, Issue 1, p. e52396
Machado-Joseph disease (MJD) or spinocerebellar ataxia type 3 (SCA3) is an autosomal dominantly-inherited neurodegenerative disorder caused by the... 
NUCLEAR-LOCALIZATION | EXPANDED POLYGLUTAMINE | CEREBELLAR DYSFUNCTION | SPINOCEREBELLAR ATAXIA | CAG REPEATS | MULTIDISCIPLINARY SCIENCES | POLYGLUTAMINE-INDUCED NEURODEGENERATION | MOUSE MODEL | INTRANUCLEAR INCLUSIONS | HUNTINGTONS-DISEASE | RAT MODEL | Locomotion - genetics | Ataxin-3 | Humans | Nerve Tissue Proteins - deficiency | Machado-Joseph Disease - complications | Repressor Proteins - deficiency | Nuclear Proteins - deficiency | Anxiety - complications | Nuclear Proteins - genetics | Machado-Joseph Disease - pathology | Exploratory Behavior | Mice, Inbred C57BL | Gene Silencing | Repressor Proteins - genetics | Mice, Transgenic | Nerve Tissue Proteins - genetics | Intranuclear Inclusion Bodies - pathology | Motor Activity - genetics | Animals | Alleles | Machado-Joseph Disease - physiopathology | Mice | Mutation | Purkinje Cells - pathology | Machado-Joseph Disease - genetics | Nervous system diseases | Genetic aspects | Genetic engineering | Viral genetics | Cerebellar ataxia | Analysis | Cerebellum | Huntingtons disease | Brain | Neurosciences | Calbindin | Gait | Neuropathology | Pathogenesis | Biology | Proteins | Ataxin | Neurodegeneration | Transgenic animals | Rodents | Inclusion bodies | Ataxia | Trinucleotide repeat diseases | Quantitative analysis | Polyglutamine | Neurodegenerative diseases | RNA-mediated interference | Exploratory behavior | Abnormalities | Medical treatment | Transgenic mice | Trinucleotide repeats | Ribonucleic acid--RNA | Activity patterns | Thickness | Gene silencing | Machado-Joseph disease | Brain research | Pharmacy | DARPP-32 protein | Immunoreactivity | RNA | Ribonucleic acid
Journal Article
Brain (London, England : 1878), ISSN 0006-8950, 11/2015, Volume 138, Issue 11, pp. 3316 - 3326
The spinocerebellar ataxias types 2 (SCA2) and 3 (SCA3) are autosomal dominantly inherited cerebellar ataxias which are caused by CAG trinucleotide repeat... 
SPINOCEREBELLAR ATAXIA TYPE-2 | RESPONSIVE PARKINSONISM | substantia nigra | DOMINANT CEREBELLAR ATAXIAS | parkinsonism | CONFIRMATION | PARCELLATION | CLINICAL-FEATURES | subthalamic nucleus | MACHADO-JOSEPH-DISEASE | spinocerebellar ataxias | DEEP BRAIN-STIMULATION | PET | NEUROSCIENCES | CLINICAL NEUROLOGY | Dopamine Plasma Membrane Transport Proteins - metabolism | Substantia Nigra - pathology | Dopaminergic Neurons - pathology | Parkinsonian Disorders - complications | Humans | Middle Aged | Machado-Joseph Disease - diagnostic imaging | Male | Positron-Emission Tomography | Substantia Nigra - metabolism | Case-Control Studies | Ataxin-2 - genetics | Young Adult | Dopaminergic Neurons - diagnostic imaging | Machado-Joseph Disease - complications | Dopaminergic Neurons - metabolism | Parkinsonian Disorders - diagnostic imaging | Aged, 80 and over | Adult | Female | Neostriatum - metabolism | Machado-Joseph Disease - pathology | Spinocerebellar Ataxias - genetics | Neostriatum - pathology | Spinocerebellar Ataxias - complications | Parkinson Disease - diagnostic imaging | Spinocerebellar Ataxias - diagnostic imaging | Repressor Proteins - genetics | Spinocerebellar Ataxias - pathology | Trinucleotide Repeat Expansion | Substantia Nigra - diagnostic imaging | Aged | Neostriatum - diagnostic imaging | Ataxin-3 - genetics | Machado-Joseph Disease - genetics
Journal Article
Neurology, ISSN 1526-632X, 2008, Volume 71, Issue 13, pp. 982 - 989
OBJECTIVE: To identify factors that determine disease severity and clinical phenotype of the most common spinocerebellar ataxias (SCAs), we studied 526... 
CAG REPEAT | GERMAN KINDREDS | GENOTYPE | FAMILIES | SCA1 | MACHADO-JOSEPH-DISEASE | EXPANSION | PHENOTYPE | CLINICAL-FEATURES | DOMINANT CEREBELLAR-ATAXIA | CLINICAL NEUROLOGY
Journal Article
Journal Article
Neurology, ISSN 0028-3878, 2011, Volume 77, Issue 11, pp. 1035 - 1041
Journal Article