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Titolo:
HUNTINGTIN IMMUNOREACTIVITY IN THE RAT NEOSTRIATUM - DIFFERENTIAL ACCUMULATION IN PROJECTION AND INTERNEURONS
Autore:
KOSINSKI CM; CHA JH; YOUNG AB; PERSICHETTI F; MACDONALD M; GUSELLA JF; PENNEY JB; STANDAERT DG;
Indirizzi:
MASSACHUSETTS GEN HOSP,DEPT NEUROL,FRUIT ST BOSTON MA 02114 HARVARD UNIV,SCH MED BOSTON MA 02114 MASSACHUSETTS GEN HOSP,MOL NEUROGENET UNIT CHARLESTOWN MA 02129
Titolo Testata:
Experimental neurology
fascicolo: 2, volume: 144, anno: 1997,
pagine: 239 - 247
SICI:
0014-4886(1997)144:2<239:HIITRN>2.0.ZU;2-J
Fonte:
ISI
Lingua:
ENG
Soggetto:
DISEASE GENE HOMOLOG; IT15 PROTEIN PRODUCT; WIDESPREAD EXPRESSION; TARGETED DISRUPTION; EMBRYONIC LETHALITY; PREFERENTIAL LOSS; CAG REPEAT; NEURONS; BRAIN; LOCALIZATION;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Science Citation Index Expanded
Citazioni:
38
Recensione:
Indirizzi per estratti:
Citazione:
C.M. Kosinski et al., "HUNTINGTIN IMMUNOREACTIVITY IN THE RAT NEOSTRIATUM - DIFFERENTIAL ACCUMULATION IN PROJECTION AND INTERNEURONS", Experimental neurology, 144(2), 1997, pp. 239-247

Abstract

Huntington's disease is caused by a mutation of the gene encoding theprotein huntingtin. Features of the human disease, characterized by selective loss of neurons from the neostriatum, can be replicated in rodents by administration of excitotoxins. In both affected individuals and the rodent model, there is massive loss of striatal projection neurons with selective sparing of interneurons. Furthermore, in the humandisease the earliest evidence of striatal injury is found in striosomal regions of the striatum. The mRNA encoding huntingtin is known to be expressed by neurons throughout the brain, a distribution which doesnot account for the selective patterns of neuronal death which are observed. Using fluorescence immunocytochemistry and confocal microscopywith an antibody to huntingtin, we have observed that in rats a subset of striatal projection neurons contains dense accumulations of huntingtin immunoreactivity (HT-ir), while most neurons in the striatum contain much smaller amounts. The intensely stained neurons are concentrated within the striatal striosomes, as defined by calbindin-D-28K staining. In the matrix regions, relatively few neurons contain dense accumulations of HT-ir, and these cells always lack perikaryal staining for calbindin-D-28K. Striatal interneurons, identified by the presence of immunoreactivity for choline acetyltransferase, parvalbumin, calretinin, or neuronal nitric oxide synthase, exhibit little or no HT-ir. The paucity of HT-ir in striatal interneurons, as well as the prominenceof staining in a subset of striosomal neurons, mirrors the selective vulnerability of these different types of cells in early stages of human Huntington's disease and in rodent excitotoxic models of the disorder. Our observations suggest that mechanisms which modulate the accumulation of huntingtin may play a central role in the neuronal degeneration of Huntington's disease. (C) 1997 Academic Press.

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Documento generato il 20/09/20 alle ore 10:43:08