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Sapp E, Ge P, Aizawa H, Bird E, Penney J, Young AB, Vonsattel J-P: Evidence for a preferential loss of enkephalin immunoreactivity in the external globus pallidus in low grade Huntington's disease using high resolution image analysis. Neuroscience 1995, 64:397-404.
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Huntingtin is a cytoplasmic protein associated with vesicles in human and rat brain neurons
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Gutekunst C-A, Levey AI, Heilman CJ, Whaley WL, Yi H, Nash NR, Rees HD, Madden HH, Hersch SM: Identification and localization of huntingtin in brain and human lymphoblastoid cell lines with anti-fusion protein antibodies. Proc Natl Acad Sci U S A 1995, 92:8710-8714.
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Trottier Y, Lutz Y, Stevanin G, Imbert G, Devys D, Cancel G, Saudou F, Weber C, David G, Tora L: Cellular localization of the Huntington's disease protein and discrimination of the normal and mutated form. Nature Genet 1995, 10:104-110. Monoclonal antibodies against four regions of the protein were used to study the localization of the Huntington's disease protein. In Huntington's disease, both the normal and abnormal protein products can be demonstrated. Their distribution mirrors expression of the gene.
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Landwehrmeyer GB, McNeil SM, Dure LS, Ge P, Aizawa H, Huang Q, Ambrose CM, Duyao MP, Bird ED, Bonilla E et al.: Huntington's disease gene: regional and cellular expression in brain of normal and affected individuals. Ann Neurol 1995, 37:218-230. Using quantitative in-situ hybridization, the authors demonstrated expression of the IT15 gene in all examined brain regions without respect to their relative involvement in the Huntington's disease degenerative process.
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A receptor changes in the substantia nigra of the rat following quinolinic acid lesions in the striatum closely resemble Huntington's disease. Neuroscience 1995, 66:507-521.
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n repeats in parent-offspring pairs with Huntington disease. Am J Hum Genet 1995, 57:593-602. The authors studied 277 parent-child pairs with Huntington's disease. The phenomenon of anticipation was confirmed. In paternal transmission, the increase in CAG repeat number correlated with the size of the mutation. This relationship was not true in maternal transmission.
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Garcia Ruiz PJ, Mena MA, Sanchez Bernardos V, Diaz Neira W, Gimenez Roldan S, Benitez J, Garcia de Yebenes J: Cerebrospinal fluid homovanillic acid is reduced in untreated Huntington's disease. Clin Neuropharm 1995, 18:58-63.
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Giordani B, Berent S, Boivin MJ, Penney JB, Lehtinen S, Markel DS, Hollingsworth Z, Butterbaugh G, Hichwa RD, Gusella JF, Young AB: Longitudinal neuropsychological and genetic linkage analysis of persons at risk for Huntington's disease. Arch Neurol 1995, 52:59-64. Four groups of patients (eight each gene-positive at-risk, gene-negative at-risk, normal control and Huntington's disease) had serial neuropsychological testing over 4 years. At-risk subjects performed more poorly than controls, suggesting social or environmental effects in Huntington's disease families, but no specific abnormalities were associated with the asymptomatic gene-carrier state.
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Markel, D.S.6
Hollingsworth, Z.7
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Young, A.B.11
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Functional decline in Huntington's disease
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Feigen A, Kieburtz K, Bordwell K, Como P, Steinberg K, Sotack J, Zimmerman C, Hickey C, Orme C, Shoulson I: Functional decline in Huntington's disease. Mov Disord 1995, 10:211-214. A total of 129 Huntington's disease patients were examined serially in order to determine the rate of decline in untreated disease. Total functional capacity declined by 0.63 ± 0.75 units per year. This information will be useful in designing clinical trials of putative protective therapies.
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44
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Trial of d-alpha-tocopherol in Huntington's disease
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Peyser CE, Folstein M, Chase GA, Starkstein S, Brandt J, Cockrell JR, Bylsma F, Coyle JT, McHugh PR, Folstein SE: Trial of d-alpha-tocopherol in Huntington's disease. Am J Psychiatry 1995, 152:1771-1775.
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Reading PJ, Dunnett SB: Embryonic striatal grafts reverse the disinhibitory effects of ibotenic acid lesions of the ventral striatum. Exp Brain Res 1995, 105:76-86.
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Campbell K, Wictorin K, Bjorklund A: Neurotransmitter-related gene expression in intrastriatal striatal transplants. I. Phenotypical characterization of striatal and non-striatal graft regions. Neuroscience 1995, 64:17-33.
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47
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Neurotransmitter-related gene expression in intrastriatal striatal transplants. II. Characterization of efferent projecting graft neurons
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Campbell K, Wictorin K, Bjorklund A: Neurotransmitter-related gene expression in intrastriatal striatal transplants. II. Characterization of efferent projecting graft neurons. Neuroscience 1995, 64:35-47.
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48
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Rationale for intrastriatal grafting of striatal neuroblasts in patients with Huntington's disease
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Peschanski M, Cesaro P, Hantraye P: Rationale for intrastriatal grafting of striatal neuroblasts in patients with Huntington's disease. Neuroscience 1995, 68:273-285. The authors review the evidence that suggests intrastriatal grafting of striatal neuroblasts may be useful in Huntington's disease.
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Peschanski, M.1
Cesaro, P.2
Hantraye, P.3
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Madrazo I, Franco-Bourland RE, Castrejon H, Cuevas C, Ostrosky-Solis F: Fetal striatal homotransplantation for Huntington's disease: first two case reports. Neurol Res 1995, 17:312-315.
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Madrazo, I.1
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Castrejon, H.3
Cuevas, C.4
Ostrosky-Solis, F.5
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50
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Molecular diagnostic analysis for Huntington's disease: A prospective evaluation
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MacMillan JC, Davies P, Harper PS: Molecular diagnostic analysis for Huntington's disease: a prospective evaluation. J Neurol Neurosurg Psychiatry 1995, 58:496-498.
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MacMillan, J.C.1
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Harper, P.S.3
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Emotional and functional impact of DNA testing on patients with symptoms of Huntington's disease
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Jankovic J, Beach J, Ashizawa T: Emotional and functional impact of DNA testing on patients with symptoms of Huntington's disease. J Med Genet 1995, 32:516-518.
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Jankovic, J.1
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Exploration of the effects of predictive testing for Huntington disease on intimate relationships
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Quaid KA, Wesson MK: Exploration of the effects of predictive testing for Huntington disease on intimate relationships. Am J Med Gen 1995, 57:46-51.
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Hereditary late-onset chorea without significant dementia: Genetic evidence for substantial phenotypic variation in Huntington's disease
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Britton JW, Uitti RH, Ahlskog JE, Robinson RG, Kremer B, Hayden MR: Hereditary late-onset chorea without significant dementia: genetic evidence for substantial phenotypic variation in Huntington's disease. Neurology 1995, 45:443-447.
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Kremer, B.5
Hayden, M.R.6
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Exclusion mapping of the benign hereditary chorea gene from the Huntington's disease locus: Report of a family
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Yapijakis C, Kapaki E, Zournas C, Rentzos M, Loukopoulos D, Papageorgiou C: Exclusion mapping of the benign hereditary chorea gene from the Huntington's disease locus: report of a family. Clin Genet 1995, 47: 133-138.
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Papageorgiou, C.6
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55
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Normal CAG repeat length in the Huntington's disease gene in senile chorea
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Shinotoh H, Calne DB, Snow B, Hayward M, Kremer B Theilmann J, Hayden MR: Normal CAG repeat length In the Huntington's disease gene in senile chorea. Neurology 1994, 44:2183-2184.
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Kremer, B.5
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Hayden, M.R.7
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56
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A new mitochondrial DNA mutation associated with progressive dementia and chorea: A clinical pathological and molecular genetic study
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Nelson I, Hanna MG, Alsanjari N, Scaravilli F, Morgan-Hughes JA, Harding AE: A new mitochondrial DNA mutation associated with progressive dementia and chorea: a clinical pathological and molecular genetic study. Ann Neurol 1995, 37:400-403.
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Molecular and clinical findings in a family with dentatorubral-pallidoluysian atrophy
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Potter NT, Meyer MA, Zimmerman AW, Eisenstadt ML, Anderson IJ: Molecular and clinical findings in a family with dentatorubral-pallidoluysian atrophy. Ann Neurol 1995, 37:273-277. Fourteen affected members of a single family with dentatorubral-pallidoluysian atrophy are described. Clinical findings and molecular biology are reviewed.
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Potter, N.T.1
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Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA)
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Koide R, Ikeuchi, T Onodera, O: Unstable expansion of CAG repeat in hereditary dentatorubral-pallidoluysian atrophy (DRPLA). Nature Genet 1994, 6:9-13.
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0029007042
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Diabetes mellitus presenting as paroxysmal kinesiogenic dystonic choreoathetosis
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Clark JD, Pahwa R, Koller C Morales D: Diabetes mellitus presenting as paroxysmal kinesiogenic dystonic choreoathetosis. [Letter]. Mov Disord 1995, 10:353-355.
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Clark, J.D.1
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Alternating hemichorea in primary antiphospholipid syndrome: Evidence for contralateral striatal hypermetabolism
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Furie R, Ishikawa T, Dhawan V, Eidelberg D: Alternating hemichorea in primary antiphospholipid syndrome: evidence for contralateral striatal hypermetabolism. Neurology 1994, 44:2197-2199.
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Furie, R.1
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Sydenham's chorea: A case showing reversible striatal abnormalities on CT and MRI
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Traill Z, Pike M, Byrne J: Sydenham's chorea: a case showing reversible striatal abnormalities on CT and MRI. Dev Med Child Neurol 1995, 37:270-273.
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Choreoathetosis as an initial sign of relapsing of herpes simplex encephalitis
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Wang H-S, Kuo M-F, Huang S-C, Chou M-L: Choreoathetosis as an initial sign of relapsing of herpes simplex encephalitis. Pediatr Neurol 1994, 11:341-345.
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Pediatr Neurol
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Wang, H.-S.1
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Hemichorea due to cryptococcal meningitis
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Weeks RA, Clough CG: Hemichorea due to cryptococcal meningitis. Mov Disord 1995, 10:522.
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Mov Disord
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Neuronal loss from the subthalamic nuclei in a patient with progressive chorea
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Sinard JH, Hedreen JC: Neuronal loss from the subthalamic nuclei in a patient with progressive chorea. Mov Disord 1995, 10:305-311.
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Mov Disord
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