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Titolo:
Double-stranded RNA-dependent protein kinase, PKR, binds preferentially toHuntington's disease (HD) transcripts and is activated in HD tissue
Autore:
Peel, AL; Rao, RV; Cottrell, BA; Hayden, MR; Ellerby, LM; Bredesen, DE;
Indirizzi:
Buck Inst, Novato, CA 94945 USA Buck Inst Novato CA USA 94945Buck Inst, Novato, CA 94945 USA Univ British Columbia, Dept Med Genet, Ctr Mol Med & Therapeut, Vancouver,BC V5Z 4H4, Canada Univ British Columbia Vancouver BC Canada V5Z 4H4 uver,BC V5Z 4H4, Canada
Titolo Testata:
HUMAN MOLECULAR GENETICS
fascicolo: 15, volume: 10, anno: 2001,
pagine: 1531 - 1538
SICI:
0964-6906(20010715)10:15<1531:DRPKPB>2.0.ZU;2-2
Fonte:
ISI
Lingua:
ENG
Soggetto:
SCA1 TRANSGENIC MICE; MYOTONIC-DYSTROPHY; TRINUCLEOTIDE REPEAT; MUTANT HUNTINGTIN; LOCALIZATION; TOXICITY; NEURODEGENERATION; EXPANSIONS; ABSENCE; NUCLEAR;
Tipo documento:
Article
Natura:
Periodico
Settore Disciplinare:
Life Sciences
Citazioni:
32
Recensione:
Indirizzi per estratti:
Indirizzo: Peel, AL Buck Inst, 8001 Redwood Blvd, Novato, CA 94945 USA Buck Inst 8001Redwood Blvd Novato CA USA 94945 ato, CA 94945 USA
Citazione:
A.L. Peel et al., "Double-stranded RNA-dependent protein kinase, PKR, binds preferentially toHuntington's disease (HD) transcripts and is activated in HD tissue", HUM MOL GEN, 10(15), 2001, pp. 1531-1538

Abstract

Fourteen neurological diseases have been associated with the expansion of trinucleotide repeat regions. These diseases have been categorized into those that give rise to the translation of toxic polyglutamine proteins and those that are untranslated. Thus far, compelling evidence has not surfaced for the inclusion of a model in which a common mechanism may participate in the pathobiology of both translated and untranslated trinucleotide diseases. In these studies we show that a double-stranded RNA-binding protein, PKR,which has previously been linked to virally-induced and stress-mediated apoptosis, preferentially binds mutant huntingtin RNA transcripts immobilizedon streptavidin columns that have been incubated with human brain extracts. These studies also show, by immunodetection in tissue slices, that PKR ispresent in its activated form in both human Huntington autopsy material and brain tissue derived from Huntington yeast artificial chromosome transgenic mice. The increased immunolocalization of the activated kinase is more pronounced in areas most affected by the disease and, coupled with the RNA binding results, suggests a role for PKR activation in the disease process.

ASDD Area Sistemi Dipartimentali e Documentali, Università di Bologna, Catalogo delle riviste ed altri periodici
Documento generato il 08/04/20 alle ore 08:42:05