| scholarly article | Q13442814 |
| P356 | DOI | 10.1212/WNL.32.8.798 |
| P698 | PubMed publication ID | 6808417 |
| P2093 | author name string | Naito H | |
| Oyanagi S | |||
| P433 | issue | 8 | |
| P407 | language of work or name | English | Q1860 |
| P921 | main subject | Choreoathetosis | Q3693493 |
| P304 | page(s) | 798-807 | |
| P577 | publication date | 1982-08-01 | |
| P1433 | published in | Neurology | Q1161692 |
| P1476 | title | Familial myoclonus epilepsy and choreoathetosis: hereditary dentatorubral-pallidoluysian atrophy | |
| P478 | volume | 32 |
| Q45305978 | A South African mixed ancestry family with Huntington disease-like 2: clinical and genetic features |
| Q35865845 | A pathogenetic classification of hereditary ataxias: is the time ripe? |
| Q73173842 | A pediatric patient with sporadic dentatorubral pallidoluysian atrophy |
| Q36894776 | A sporadic case of dentatorubral pallidoluysian atrophy (DRPLA) with CAG repeat expansion but no clinical abnormalities in the father |
| Q24324461 | Abnormal gene product identified in hereditary dentatorubral–pallidoluysian atrophy (DRPLA) brain |
| Q34386791 | Advances in the genetics of progressive myoclonus epilepsy |
| Q71999906 | Anticipation in hereditary dentatorubral-pallidoluysian atrophy |
| Q34192381 | Anticipation, imprinting, trinucleotide repeat expansions and psychoses |
| Q47071965 | Atrophin contributes to the negative regulation of epidermal growth factor receptor signaling in Drosophila |
| Q24310521 | Atrophin-1, the DRPLA gene product, interacts with two families of WW domain-containing proteins |
| Q21202869 | Autosomal dominant cerebellar ataxia type I: a review of the phenotypic and genotypic characteristics |
| Q45289505 | Autosomal-dominant dentatorubropallidoluysian atrophy in the United Kingdom |
| Q28589500 | C-terminal deletion of the atrophin-1 protein results in growth retardation but not neurodegeneration in mice |
| Q34677190 | CAG repeat disorder models and human neuropathology: similarities and differences |
| Q34228083 | Cervical dystonia in dentatorubral-pallidoluysian atrophy. |
| Q48960065 | Chapter 47: history of clinical neurology in Japan |
| Q48821379 | Choreiform movements in spinocerebellar ataxia type 1. |
| Q58417281 | Classification of progressive myoclonus epilepsies and related disorders |
| Q35594577 | Classification of the myoclonic epilepsies |
| Q41527046 | Clinical Aspects of CAG Repeat Diseases |
| Q48479313 | Clinical and electroencephalographic findings in juvenile type DRPLA. |
| Q71853048 | Clinicopathological features of MERRF |
| Q41364983 | Clinicopathology of spinocerebellar degeneration: its correlation to the unstable CAG repeat of the affected gene |
| Q34306449 | Computational analysis of calcium signaling and membrane electrophysiology in cerebellar Purkinje neurons associated with ataxia |
| Q36981725 | Delirium associated with Joseph disease |
| Q33620531 | Dentato-rubro-pallido-luysian atrophy: a clinico-pathological study |
| Q41557473 | Dentatorubral and pallidoluysian atrophy (DRPLA). Clinical and neuropathological findings in genetically confirmed North American and European pedigrees |
| Q28250966 | Dentatorubral and pallidoluysian atrophy expansion of an unstable CAG trinucleotide on chromosome 12p |
| Q48330208 | Dentatorubral-pallidoluysian atrophy (DRPLA) with a small ganglioglioma component containing neurofibrillary tangles and polyglutamine aggregation. |
| Q48186729 | Dentatorubral-pallidoluysian atrophy (DRPLA): The 50th Anniversary of Japanese Society of Neuropathology |
| Q48095256 | Differences in evoked potential characteristics between DRPLA patients and patients with progressive myoclonic epilepsy: preliminary findings indicating usefulness for differential diagnosis |
| Q38792714 | Discovery of Therapeutic Approaches for Polyglutamine Diseases: A Summary of Recent Efforts |
| Q35589292 | Dominant spinocerebellar ataxias: a molecular approach to classification, diagnosis, pathogenesis and the future |
| Q47071704 | Drosophila atrophin homolog functions as a transcriptional corepressor in multiple developmental processes |
| Q44811145 | Early-childhood progressive myoclonus epilepsy presenting as partial seizures in dentatorubral-pallidoluysian atrophy |
| Q46491267 | Electroclinical features of epilepsy in patients with juvenile type dentatorubral-pallidoluysian atrophy |
| Q43777521 | Electronystagmographic findings in patients with cerebral degenerative disease. |
| Q45288856 | Elongated CAG repeats of the B37 gene in a Danish family with dentato-rubro-pallido-luysian atrophy. |
| Q34836189 | Epigenetic regulation of Atrophin1 by lysine-specific demethylase 1 is required for cortical progenitor maintenance |
| Q33597183 | Exclusion mapping of the hereditary dentatorubropallidoluysian atrophy gene from the Huntington's disease locus |
| Q48795581 | Expression and distribution of the dentatorubral-pallidoluysian atrophy gene product (atrophin-1/drplap) in neuronal and non-neuronal tissues |
| Q48758760 | Expression of dentatorubral-pallidoluysian atrophy (DRPLA) proteins in patients |
| Q73161798 | Familial benign myoclonus epilepsy of adult onset: a previously unrecognized myoclonic disorder |
| Q38896237 | Fragile X-associated tremor/ataxia syndrome: phenotypic comparisons with other movement disorders |
| Q28190085 | Genomic structure and alternative splicing of the insulin receptor tyrosine kinase substrate of 53-kDa protein |
| Q35058863 | Genotype-phenotype correlation in CAG-repeat diseases |
| Q44424128 | Glyceraldehyde 3‐phosphate dehydrogenase and endothelin‐1 immunoreactivity is associated with cerebral white matter damage in dentatorubral–pallidoluysian atrophy |
| Q78351738 | Head tremor in dentatorubral-pallidoluysian atrophy |
| Q34063851 | Hereditary dentatorubral-pallidoluysian atrophy |
| Q41626530 | Hereditary epilepsy syndromes. |
| Q35889330 | Huntington disease without CAG expansion: phenocopies or errors in assignment? |
| Q41527116 | Huntington's Disease and Dentatorubral‐Pallidoluysian Atrophy: Proteins, Pathogenesis and Pathology |
| Q39625800 | Importance of CAG repeat length in childhood-onset dentatorubral-pallidoluysian atrophy |
| Q48147203 | Long-term disability and prognosis in dentatorubral-pallidoluysian atrophy: a correlation with CAG repeat length |
| Q35643880 | Mapping of the gene for Machado-Joseph disease within a 3.6-cM interval flanked by D14S291/D14S280 and D14S81, on the basis of studies of linkage and linkage disequilibrium in 24 Japanese families. |
| Q50584350 | Maternally inherited mitochondrial myopathy and myoclonic epilepsy. |
| Q52210236 | Molecular and clinical findings in a family with dentatorubral-pallidoluysian atrophy. |
| Q24671945 | Molecular cloning of a full-length cDNA for dentatorubral-pallidoluysian atrophy and regional expressions of the expanded alleles in the CNS |
| Q24676632 | Molecular pathology of dentatorubral-pallidoluysian atrophy |
| Q45291542 | Molecular re-investigation of patients with Huntington's disease in Wessex reveals a family with dentatorubral and pallidoluysian atrophy |
| Q52703722 | Neurodegeneration by polyglutamine Atrophin is not rescued by induction of autophagy. |
| Q34100009 | Neuronal nuclear alterations in dentatorubral-pallidoluysian atrophy: ultrastructural and morphometric studies of the cerebellar granule cells |
| Q48152885 | Neuropathology of myoclonus epilepsy associated with ragged-red fibers (Fukuhara's disease). |
| Q35881812 | Non-Mendelian transmission in dentatorubral-pallidoluysian atrophy and Machado-Joseph disease: the mutant allele is preferentially transmitted in male meiosis |
| Q42950804 | Normal CAG repeat variation at the DRPLA locus in world populations |
| Q33890266 | Nuclear accumulation of truncated atrophin-1 fragments in a transgenic mouse model of DRPLA. |
| Q28593520 | Nuclear localization of a non-caspase truncation product of atrophin-1, with an expanded polyglutamine repeat, increases cellular toxicity |
| Q48459242 | Oligodendrocytic polyglutamine pathology in dentatorubral-pallidoluysian atrophy |
| Q34185428 | Pathological accumulation of atrophin-1 in dentatorubralpallidoluysian atrophy |
| Q38107677 | Pharmacological protein targets in polyglutamine diseases: mutant polypeptides and their interactors |
| Q38210865 | Polyglutamine (PolyQ) diseases: genetics to treatments. |
| Q36589169 | Polyglutamine disease: recent advances in the neuropathology of dentatorubral-pallidoluysian atrophy |
| Q54752346 | Portuguese families with dentatorubropallidoluysian atrophy (DRPLA) share a common haplotype of Asian origin. |
| Q41255582 | Precise chromosomal locations of the genes for dentatorubral-pallidoluysian atrophy (DRPLA), von Willebrand factor (F8vWF) and parathyroid hormone-like hormone (PTHLH) in human chromosome 12p by deletion mapping |
| Q36075070 | Progressive myoclonic epilepsies: a review of genetic and therapeutic aspects |
| Q51007488 | Progressive myoclonus epilepsy: extraneuronal brown pigment deposition and system neurodegeneration in the brains of Japanese patients with novel SCARB2 mutations. |
| Q39639663 | Proteolytic processing regulates pathological accumulation in dentatorubral-pallidoluysian atrophy. |
| Q85238382 | Radiologic and Neuropathologic Findings in Patients in a Family with Dentatorubral-Pallidoluysian Atrophy |
| Q34181237 | Ramsay Hunt syndrome and coeliac disease: a new association? |
| Q48378973 | Recent advances in dementia research in Japan: non-Alzheimer-type degenerative dementias |
| Q48470466 | Selective colocalization of transglutaminase-like activity in ubiquitinated intranuclear inclusions of hereditary dentatorubral-pallidoluysian atrophy |
| Q37090117 | Severe neurological phenotypes of Q129 DRPLA transgenic mice serendipitously created by en masse expansion of CAG repeats in Q76 DRPLA mice. |
| Q45148750 | Sharing of polyglutamine localization by the neuronal nucleus and cytoplasm in CAG-repeat diseases |
| Q30725562 | Single cell analysis of CAG repeat in brains of dentatorubral-pallidoluysian atrophy (DRPLA). |
| Q46890244 | Sodium butyrate ameliorates histone hypoacetylation and neurodegenerative phenotypes in a mouse model for DRPLA. |
| Q42037871 | Somatic mosaicism of expanded CAG repeats in brains of patients with dentatorubral-pallidoluysian atrophy: cellular population-dependent dynamics of mitotic instability |
| Q48524084 | Somatic mosaicism of the expanded CAG trinucleotide repeat in mRNAs for the responsible gene of Machado-Joseph disease (MJD), dentatorubral-pallidoluysian atrophy (DRPLA), and spinal and bulbar muscular atrophy (SBMA). |
| Q28306588 | Structure and expression of the gene responsible for the triplet repeat disorder, dentatorubral and pallidoluysian atrophy (DRPLA) |
| Q55016029 | Successful Treatment with Olanzapine of Psychosis in Dentatorubral-pallidoluysian Atrophy: A Case Report. |
| Q48537185 | Suppression of aggregate formation and apoptosis by transglutaminase inhibitors in cells expressing truncated DRPLA protein with an expanded polyglutamine stretch. |
| Q41527072 | The CAG/polyglutamine tract diseases: gene products and molecular pathogenesis |
| Q34680311 | The Huntington's disease-like syndromes: what to consider in patients with a negative Huntington's disease gene test |
| Q41527060 | The Neuropathology of CAG Repeat Diseases: Review and Update of Genetic and Molecular Features |
| Q38221489 | The Ramsay Hunt syndrome is a useful clinical entity |
| Q38952789 | The history of progressive myoclonus epilepsies |
| Q35340099 | The role for alterations in neuronal activity in the pathogenesis of polyglutamine repeat disorders |
| Q49019300 | Time course of polyglutamine aggregate body formation and cell death: enhanced growth in nucleus and an interval for cell death |
| Q35145859 | Transcriptional derepression as a cause of genetic diseases |
| Q40654052 | Trinucleotide repeat expansion in neurological disease |
| Q72312857 | Triplet repeats strike again |
| Q48697027 | Two different pathological conditions of photoparoxysmal responses in hereditary dentatorubral-pallidoluysian atrophy |
| Q28250990 | Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA) |
| Q33934498 | Widespread occurrence of intranuclear atrophin-1 accumulation in the central nervous system neurons of patients with dentatorubral-pallidoluysian atrophy |
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