| scholarly article | Q13442814 |
| review article | Q7318358 |
| P356 | DOI | 10.1016/S0072-9752(07)01256-0 |
| P698 | PubMed publication ID | 18631782 |
| P2093 | author name string | Christian Keller | |
| Jean Paul G Vonsattel | |||
| Maria Del Pilar Amaya | |||
| P2860 | cites work | A huntingtin-associated protein enriched in brain with implications for pathology | Q24303499 |
| Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons | Q24317574 | ||
| Inhibition of caspase-1 slows disease progression in a mouse model of Huntington's disease | Q28114818 | ||
| The Haw River syndrome: dentatorubropallidoluysian atrophy (DRPLA) in an African-American family | Q28240104 | ||
| Formation of neuronal intranuclear inclusions underlies the neurological dysfunction in mice transgenic for the HD mutation | Q28246858 | ||
| Dentatorubral and pallidoluysian atrophy expansion of an unstable CAG trinucleotide on chromosome 12p | Q28250966 | ||
| Long glutamine tracts cause nuclear localization of a novel form of huntingtin in medium spiny striatal neurons in HdhQ92 and HdhQ111 knock-in mice | Q28505664 | ||
| Inactivation of the mouse Huntington's disease gene homolog Hdh | Q28509662 | ||
| Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form | Q28509836 | ||
| Huntingtin is required for neurogenesis and is not impaired by the Huntington's disease CAG expansion | Q28594526 | ||
| The functional anatomy of basal ganglia disorders | Q29617461 | ||
| Aggregation of huntingtin in neuronal intranuclear inclusions and dystrophic neurites in brain | Q29617982 | ||
| Functional architecture of basal ganglia circuits: neural substrates of parallel processing | Q29618729 | ||
| Expanded CAG repeats in exon 1 of the Huntington's disease gene stimulate dopamine-mediated striatal neuron autophagy and degeneration | Q30992818 | ||
| Neuronal distribution in the putamen in Huntington's disease | Q33621030 | ||
| The relevance of the Lewy body to the pathogenesis of idiopathic Parkinson's disease | Q33631292 | ||
| Differential loss of striatal projection neurons in Huntington disease | Q33637273 | ||
| Identification and localization of huntingtin in brain and human lymphoblastoid cell lines with anti-fusion protein antibodies | Q33920421 | ||
| Widespread occurrence of intranuclear atrophin-1 accumulation in the central nervous system neurons of patients with dentatorubral-pallidoluysian atrophy | Q33934498 | ||
| Energetics in the pathogenesis of neurodegenerative diseases | Q33944937 | ||
| Transcriptional dysregulation in Huntington's disease | Q34001953 | ||
| Huntington disease phenocopy is a familial prion disease | Q34044888 | ||
| A repeat expansion in the gene encoding junctophilin-3 is associated with Huntington disease-like 2. | Q34100492 | ||
| Huntington's disease: the challenge for cell biologists | Q34106564 | ||
| Localization of the gene for a novel autosomal recessive neurodegenerative Huntington-like disorder to 4p15.3. | Q34145047 | ||
| Evidence for degenerative and regenerative changes in neostriatal spiny neurons in Huntington's disease | Q34168124 | ||
| Huntington's disease in children. Neuropathologic study of four cases | Q34205152 | ||
| Replication of the neurochemical characteristics of Huntington's disease by quinolinic acid | Q34384772 | ||
| A detailed examination of substance P in pathologically graded cases of Huntington's disease | Q45295316 | ||
| Huntingtin localization in brains of normal and Huntington's disease patients | Q45295341 | ||
| Linkage to the Huntington's disease locus in a family with unusual clinical and pathological features | Q45295703 | ||
| Neuronal and glial somal size in the prefrontal cortex: a postmortem morphometric study of schizophrenia and Huntington disease | Q45295751 | ||
| Neuronal distribution of intranuclear inclusions in Huntington's disease with adult onset. | Q45296154 | ||
| Intranuclear neuronal inclusions in Huntington's disease and dentatorubral and pallidoluysian atrophy: correlation between the density of inclusions and IT15 CAG triplet repeat length | Q45296163 | ||
| Behavioural abnormalities and selective neuronal loss in HD transgenic mice expressing mutated full-length HD cDNA. | Q45296558 | ||
| Somatostatin and neuropeptide Y concentrations in pathologically graded cases of Huntington's disease | Q45297003 | ||
| Regional specificity of brain atrophy in Huntington's disease. | Q45297061 | ||
| Selective sparing of a class of striatal neurons in Huntington's disease | Q45297134 | ||
| Monozygotic twins discordant for Huntington disease after 7 years | Q45297153 | ||
| Neuropathological classification of Huntington's disease | Q45297167 | ||
| Huntington's disease in Venezuela: neurologic features and functional decline | Q45297315 | ||
| Axonal transport of N-terminal huntingtin suggests early pathology of corticostriatal projections in Huntington disease | Q45297318 | ||
| Nuclear and neuropil aggregates in Huntington's disease: relationship to neuropathology. | Q45297497 | ||
| Morphologic and histochemical characteristics of a spared subset of striatal neurons in Huntington's disease | Q45298031 | ||
| Regional cortical thinning in preclinical Huntington disease and its relationship to cognition | Q45298420 | ||
| Sparing of acetylcholinesterase-containing striatal neurons in Huntington's disease | Q45298464 | ||
| A YAC mouse model for Huntington's disease with full-length mutant huntingtin, cytoplasmic toxicity, and selective striatal neurodegeneration. | Q45298582 | ||
| Amyloid formation by mutant huntingtin: threshold, progressivity and recruitment of normal polyglutamine proteins | Q45298596 | ||
| Morphometric demonstration of atrophic changes in the cerebral cortex, white matter, and neostriatum in Huntington's disease | Q45299064 | ||
| Dementia of the Alzheimer's type and Huntington's disease | Q45299681 | ||
| The pattern of neurodegeneration in Huntington's disease: a comparative study of cannabinoid, dopamine, adenosine and GABA(A) receptor alterations in the human basal ganglia in Huntington's disease | Q45300428 | ||
| Reduced Purkinje cell density in Huntington's disease | Q45304060 | ||
| Selective loss of striatal preprotachykinin neurons in a phenocopy of Huntington's disease | Q45305678 | ||
| Cerebellar atrophy in Huntington's disease | Q45306506 | ||
| Evidence for a preferential loss of enkephalin immunoreactivity in the external globus pallidus in low grade Huntington's disease using high resolution image analysis | Q45307235 | ||
| Abnormally high neuronal density in the schizophrenic cortex. A morphometric analysis of prefrontal area 9 and occipital area 17. | Q45307345 | ||
| CAG expansion affects the expression of mutant Huntingtin in the Huntington's disease brain | Q45307352 | ||
| Reduced expression of preproenkephalin in striatal neurons from huntington's disease patients | Q45307627 | ||
| The neostriatal mosaic: II. Patch- and matrix-directed mesostriatal dopaminergic and non-dopaminergic systems. | Q48180155 | ||
| Mutant huntingtin expression in clonal striatal cells: dissociation of inclusion formation and neuronal survival by caspase inhibition. | Q48298684 | ||
| Do defecs in mitochondrial energy metabolism underlie the pathology of neurodegenerative diseases? | Q48317832 | ||
| Dentatorubropallidoluysian atrophy: clinicopathological study of dementia and involvement of the nucleus basalis of Meynert in seven autopsy cases | Q48342240 | ||
| Huntingtin acts in the nucleus to induce apoptosis but death does not correlate with the formation of intranuclear inclusions. | Q48373564 | ||
| Huntington disease | Q48465200 | ||
| A Golgi study of the human neostriatum: neurons and afferent fibers | Q48496147 | ||
| 'Haw River Syndrome' or Dentato-Rubro-Pallido-Luysian Atrophy? | Q48555296 | ||
| The aging brain. Limitations in our knowledge and future approaches | Q48613202 | ||
| Neurochemical architecture of the human striatum | Q48656419 | ||
| Heterogeneous topographic and cellular distribution of huntingtin expression in the normal human neostriatum. | Q48723938 | ||
| A new hypothesis for dystonia | Q48830697 | ||
| Profound loss of layer II entorhinal cortex neurons occurs in very mild Alzheimer's disease. | Q48963534 | ||
| Cytoarchitectonic heterogeneity of the primate neostriatum: subdivision into Island and Matrix cellular compartments | Q48967207 | ||
| Concentric hyalin intraneuronal inclusions of Lewy type in the brains of elderly persons (50 incidental cases): relationship to parkinsonism | Q49024553 | ||
| Cerebral amyloid deposition and diffuse plaques in "normal" aging: Evidence for presymptomatic and very mild Alzheimer's disease | Q49103500 | ||
| Neuronal types in the striatum of man | Q49116254 | ||
| Ataxia, chorea, seizures, and dementia. Pathologic features of a newly defined familial disorder. | Q54367447 | ||
| Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 2-1992. Chorea and progressive dementia in an 88-year-old woman | Q68052105 | ||
| Neurochemical and histologic characterization of striatal excitotoxic lesions produced by the mitochondrial toxin 3-nitropropionic acid | Q72098398 | ||
| Activity of pallidal neurons in the monkey during dyskinesia induced by injection of bicuculline in the external pallidum | Q72244709 | ||
| Polyglutamine pathogenesis: emergence of unifying mechanisms for Huntington's disease and related disorders | Q34952853 | ||
| Glutamine repeats as polar zippers: their possible role in inherited neurodegenerative diseases | Q35442097 | ||
| Huntingtin-protein interactions and the pathogenesis of Huntington's disease | Q35704403 | ||
| Neurotransmitters and neuromodulators in the basal ganglia | Q36586002 | ||
| Venezuelan kindreds reveal that genetic and environmental factors modulate Huntington's disease age of onset | Q37073913 | ||
| Third Dorothy S. Russell memorial lecture. Huntington's disease: some recent neuropathological studies | Q37868100 | ||
| Preferential loss of preproenkephalin versus preprotachykinin neurons from the striatum of Huntington's disease patients | Q38288534 | ||
| Bilateral projections from precentral motor cortex to the putamen and other parts of the basal ganglia. An autoradiographic study in Macaca fascicularis | Q39336189 | ||
| The rigid form of Huntington's disease | Q39408751 | ||
| The morphology of Marinesco bodies (paranucleolar corpuscles) in the melanin-pigmented nuclei of the brain-stem | Q39414998 | ||
| Morphometric studies of the neuropathological changes in choreatic diseases | Q39776066 | ||
| Molecular genetics of Alzheimer disease: identification of genes and gene mutations | Q40517536 | ||
| Diagnosis and evaluation of dementia | Q40591535 | ||
| Transglutaminase cross-links in intranuclear inclusions in Huntington disease | Q40677519 | ||
| Striatal volume loss in HD as measured by MRI and the influence of CAG repeat | Q40684835 | ||
| The basal ganglia and adaptive motor control | Q40706589 | ||
| Huntingtin is present in the nucleus, interacts with the transcriptional corepressor C-terminal binding protein, and represses transcription | Q40764541 | ||
| Hirano bodies and related neuronal inclusions | Q40765085 | ||
| A Golgi study of neuronal types in the neostriatum of monkeys | Q40887852 | ||
| Mutant huntingtin forms in vivo complexes with distinct context-dependent conformations of the polyglutamine segment | Q40922131 | ||
| Metabotropic glutamate receptors: a new target for the therapy of neurodegenerative disorders? | Q41107509 | ||
| Striatal and nigral neuron subpopulations in rigid Huntington's disease: implications for the functional anatomy of chorea and rigidity-akinesia | Q41174270 | ||
| Neurons containing NADPH-diaphorase are selectively resistant to quinolinate toxicity | Q41342466 | ||
| Cortico-striate projections in the rhesus monkey: The organization of certain cortico-caudate connections | Q41353089 | ||
| Projections from the primary somatosensory cortex to basal ganglia and thalamus in the monkey | Q41354219 | ||
| The Neuropathology of CAG Repeat Diseases: Review and Update of Genetic and Molecular Features | Q41527060 | ||
| Toward understanding the molecular pathology of Huntington's disease. | Q41527106 | ||
| Dentatorubral and pallidoluysian atrophy (DRPLA). Clinical and neuropathological findings in genetically confirmed North American and European pedigrees | Q41557473 | ||
| Protein fate in neurodegenerative proteinopathies: polyglutamine diseases join the (mis)fold | Q41763638 | ||
| Distribution of inclusions in neuronal nuclei and dystrophic neurites in Huntington disease brain | Q42468252 | ||
| Functional anatomy of the basal ganglia in X-linked recessive dystonia-parkinsonism | Q42478674 | ||
| Survival of basal ganglia neuropeptide Y-somatostatin neurones in Huntington's disease | Q42503175 | ||
| Decreased neuronal and increased oligodendroglial densities in Huntington's disease caudate nucleus | Q43814253 | ||
| Neuropathological background of twenty-seven centenarian brains | Q44991568 | ||
| Huntington's disease gene: regional and cellular expression in brain of normal and affected individuals | Q45288801 | ||
| Hereditary late-onset chorea without significant dementia: genetic evidence for substantial phenotypic variation in Huntington's disease | Q45288982 | ||
| Huntington disease associated with Alzheimer disease | Q45289814 | ||
| A worldwide study of the Huntington's disease mutation. The sensitivity and specificity of measuring CAG repeats | Q45289978 | ||
| HUNTINGTON'S CHOREA IN CHILDHOOD. | Q45290802 | ||
| OBSERVATIONS ON HUNTINGTON'S CHOREA IN CHILDHOOD. | Q45290942 | ||
| Predictors of neuropathological severity in 100 patients with Huntington's disease | Q45291219 | ||
| Selective neurodegeneration in Huntington's disease | Q45291416 | ||
| Relationship between trinucleotide repeats and neuropathological changes in Huntington's disease | Q45291624 | ||
| Mitochondrial defect in Huntington's disease caudate nucleus | Q45291734 | ||
| Allocortical involvement in Huntington's disease | Q45291757 | ||
| Huntingtin and DRPLA proteins selectively interact with the enzyme GAPDH. | Q45291767 | ||
| Locus coeruleus involvement in Huntington's disease | Q45291838 | ||
| Striatal D1 and D2 dopamine receptor loss in asymptomatic mutation carriers of Huntington's disease | Q45292176 | ||
| Morphometric analysis of the prefrontal cortex in Huntington's disease | Q45293165 | ||
| The hypothalamic lateral tuberal nucleus and the characteristics of neuronal loss in Huntington's disease | Q45293493 | ||
| Neuronal loss in layers V and VI of cerebral cortex in Huntington's disease | Q45293583 | ||
| Cortical peptide changes in Huntington's disease may be independent of striatal degeneration | Q45294154 | ||
| Degeneration of pyramidal projection neurons in Huntington's disease cortex | Q45294227 | ||
| CAG repeat number governs the development rate of pathology in Huntington's disease | Q45294278 | ||
| Huntington's disease CAG trinucleotide repeats in pathologically confirmed post-mortem brains | Q45294391 | ||
| P921 | main subject | Huntington's disease | Q190564 |
| P304 | page(s) | 599-618 | |
| P577 | publication date | 2008-01-01 | |
| P1433 | published in | Handbook of clinical neurology / edited by P.J. Vinken and G.W. Bruyn | Q26842295 |
| P1476 | title | Neuropathology of Huntington's disease. | |
| P478 | volume | 89 |
| Q39682358 | A Grey Box Neural Network Model of Basal Ganglia for Gait Signal of Patients with Huntington Disease |
| Q35087700 | A monoclonal antibody TrkB receptor agonist as a potential therapeutic for Huntington's disease |
| Q41021109 | AAV1/2-mediated BDNF gene therapy in a transgenic rat model of Huntington's disease |
| Q41840788 | Abnormal Visual Scanning of Emotionally Evocative Natural Scenes in Huntington's Disease. |
| Q38986023 | Altered expression of 3-betahydroxysterol delta-24-reductase/selective Alzheimer's disease indicator-1 gene in Huntington's disease models. |
| Q49560971 | Early Alterations in Operant Performance and Prominent Huntingtin Aggregation in a Congenic F344 Rat Line of the Classical CAGn51trunc Model of Huntington Disease |
| Q37488273 | Emerging drug therapies in Huntington's disease |
| Q34495971 | Estimating premorbid functioning in huntington's disease: the relationship between disease progression and the wide range achievement test reading subtest. |
| Q36300780 | Further investigation of phenotypes and confounding factors of progressive ratio performance and feeding behavior in the BACHD rat model of Huntington disease |
| Q88805262 | Genetic load determines atrophy in hand cortico-striatal pathways in presymptomatic Huntington's disease |
| Q42714345 | Induced pluripotent stem cells from patients with Huntington's disease show CAG-repeat-expansion-associated phenotypes |
| Q45299568 | MRI measures of corpus callosum iron and myelin in early Huntington's disease. |
| Q42654161 | Optimization of trans-Splicing for Huntington's Disease RNA Therapy |
| Q45305834 | Reduced cell size, chromosomal aberration and altered proliferation rates are characteristics and confounding factors in the STHdh cell model of Huntington disease. |
| Q96230762 | Shedding a new light on Huntington's disease: how blood can both propagate and ameliorate disease pathology |
| Q37465080 | Somatic increase of CCT8 mimics proteostasis of human pluripotent stem cells and extends C. elegans lifespan |
| Q90682346 | Spectrum of tau pathologies in Huntington's disease |
| Q38663086 | The Complexity of Clinical Huntington's Disease: Developments in Molecular Genetics, Neuropathology and Neuroimaging Biomarkers |
| Q38223504 | The role of dopamine in Huntington's disease |
| Q35558091 | The role of the amygdala during emotional processing in Huntington's disease: from pre-manifest to late stage disease |
| Q58414022 | The ubiquitin ligase UBR5 suppresses proteostasis collapse in pluripotent stem cells from Huntington's disease patients |
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