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Titolo:
DOPAMINE TRANSPORTERS ARE MARKEDLY REDUCED IN LESCH-NYHAN DISEASE IN-VIVO
Autore:
WONG DF; HARRIS JC; NAIDU S; YOKOI F; MARENCO S; DANNALS RF; RAVERT HT; YASTER M; EVANS A; ROUSSET O; BRYAN RN; GJEDDE A; KUHAR MJ; BREESE GR;
Indirizzi:
JOHNS HOPKINS MED INST,JOHC RES WING 3245,601 N CAROLINE ST BALTIMOREMD 21287 JOHNS HOPKINS UNIV,SCH MED,DEPT RADIOL BALTIMORE MD 21205 JOHNS HOPKINS UNIV,SCH MED,DEPT PSYCHIAT & BEHAV SCI BALTIMORE MD 21205 JOHNS HOPKINS UNIV,SCH MED,DEPT NEUROL BALTIMORE MD 21205 JOHNS HOPKINS UNIV,SCH MED,DEPT ANESTHESIOL BALTIMORE MD 21205 JOHNS HOPKINS UNIV,SCH MED,DEPT PEDIAT BALTIMORE MD 21205 KENNEDY KRIEGER INST BALTIMORE MD 21205 MONTREAL NEUROL INST,PET CTR MONTREAL PQ CANADA AARHUS UNIV HOSP,PET CTR DK-8000 AARHUS DENMARK EMORY UNIV,YERKES REG PRIMATE RES CTR ATLANTA GA 30322 UNIV N CAROLINA,DEPT PSYCHIAT CHAPEL HILL NC 27599
Titolo Testata:
Proceedings of the National Academy of Sciences of the United Statesof America
fascicolo: 11, volume: 93, anno: 1996,
pagine: 5539 - 5543
SICI:
0027-8424(1996)93:11<5539:DTAMRI>2.0.ZU;2-P
Fonte:
ISI
Lingua:
ENG
Soggetto:
COCAINE RECOGNITION SITES; BASAL GANGLIA; RETT-SYNDROME; BRAIN; CLONING; INVIVO; NEUROTRANSMITTERS; WIN-35,428; EXPRESSION; RECEPTORS;
Keywords:
MENTAL RETARDATION; POSITRON EMISSION TOMOGRAPHY; NEURODEVELOPMENT; RETT SYNDROME;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
39
Recensione:
Indirizzi per estratti:
Citazione:
D.F. Wong et al., "DOPAMINE TRANSPORTERS ARE MARKEDLY REDUCED IN LESCH-NYHAN DISEASE IN-VIVO", Proceedings of the National Academy of Sciences of the United Statesof America, 93(11), 1996, pp. 5539-5543

Abstract

Dopamine (DA) deficiency has been implicated in Lesch-Nyhan disease (LND), a genetic disorder that is characterized by hyperuricemia, choreoathetosis, dystonia, and compulsive self-injury. To establish that DAdeficiency is present in LND, the ligand WIN-35,428, which binds to DA transporters, was used to estimate the density of DA-containing neurons in the caudate and putamen of six patients with classic LND. Comparisons were made with 10 control subjects and 3 patients with Rett syndrome. Three methods were used to quantify the binding of the DA transporter so that its density could be estimated by a single dynamic positron emission tomography study. These approaches included the caudate-or putamen-to-cerebellum ratio of ligand at 80-90 min postinjection, kinetic analysis of the binding potential [B-max/(K-d . V-d)] using the assumption of equal partition coefficients in the striatum and the cerebellum and graphical analysis of the binding potential. Depending on the method of analysis, a 50-63% reduction of the binding to DA transporters in the caudate, and a 64-75% reduction in the putamen of the LND patients was observed compared to the normal control group. When LND patients were compared to Rett syndrome patients, similar reductions were found in the caudate (53-61%) and putamen (67-72%) in LND patients. Transporter binding in Rett syndrome patients was not significantly different from the normal controls. Finally, volumetric magnetic resonance imaging studies detected a 30% reduction in the caudate volumeof LND patients. To ensure that a reduction in the caudate volume would not confound the results, a rigorous partial volume correction of the caudate time activity curve was performed. This correction resultedin an even greater decrease in the caudate-cerebellar ratio in LND patients when contrasted to controls. To our knowledge, these findings provide the first in vivo documentation of a dopaminergic reduction in LND and illustrate the role of positron emission tomography imaging ininvestigating neurodevelopmental disorders.

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Documento generato il 28/11/20 alle ore 15:53:45