Matches in SemOpenAlex for { <https://semopenalex.org/work/W1998371279> ?p ?o ?g. }
Showing items 1 to 86 of
86
with 100 items per page.
- W1998371279 endingPage "A8" @default.
- W1998371279 startingPage "A8.2" @default.
- W1998371279 abstract "Huntington9s disease (HD) is a late-onset progressive neurodegenerative disorder characterised by irrepressible motor dysfunction, cognitive decline and psychiatric disturbances for which there is no effective disease-modifying treatment. The proteolytic cleavage of huntingtin (HTT) to generate N-terminal fragments has been proposed to be a key aspect of HD pathogenesis. In particular, it has been shown that HTT can be cleaved at amino acid 586 by caspase-6 (CASP6) and that prevention of cleavage at this site is neuroprotective and can rescue HD-related phenotypes in YAC transgenic HD mouse models. To further explore the role that CASP6 plays in HTT proteolysis, we have evaluated the effects of the genetic ablation of CASP6 in the HdhQ150 knock-in mouse model of HD. Here we show that the loss of CASP6 has no effect on the proteolysis of HTT, and does not modify the pattern of N-terminal HTT fragments that are present in the brains of these animals. Furthermore, we show that CASP6 ablation does not influence the steady-state levels of soluble wild type or mutant HTT in the brains of presymptomatic mice. Therefore, we conclude that CASP6 inhibition does not modify HTT proteolysis in the HdhQ150 mouse model of HD, and that targeting CASP6 as a therapeutic strategy should be approached with caution in the context of this complex disease." @default.
- W1998371279 created "2016-06-24" @default.
- W1998371279 creator A5013385350 @default.
- W1998371279 creator A5020296130 @default.
- W1998371279 creator A5030906053 @default.
- W1998371279 creator A5033506721 @default.
- W1998371279 creator A5041271209 @default.
- W1998371279 creator A5056951529 @default.
- W1998371279 date "2012-08-29" @default.
- W1998371279 modified "2023-09-27" @default.
- W1998371279 title "B09 Caspase-6 does not contribute to the proteolysis of mutant huntingtin in the HDHQ150 knock-in mouse model of Huntington's disease" @default.
- W1998371279 doi "https://doi.org/10.1136/jnnp-2012-303524.25" @default.
- W1998371279 hasPublicationYear "2012" @default.
- W1998371279 type Work @default.
- W1998371279 sameAs 1998371279 @default.
- W1998371279 citedByCount "0" @default.
- W1998371279 crossrefType "journal-article" @default.
- W1998371279 hasAuthorship W1998371279A5013385350 @default.
- W1998371279 hasAuthorship W1998371279A5020296130 @default.
- W1998371279 hasAuthorship W1998371279A5030906053 @default.
- W1998371279 hasAuthorship W1998371279A5033506721 @default.
- W1998371279 hasAuthorship W1998371279A5041271209 @default.
- W1998371279 hasAuthorship W1998371279A5056951529 @default.
- W1998371279 hasConcept C102230213 @default.
- W1998371279 hasConcept C104317684 @default.
- W1998371279 hasConcept C141035611 @default.
- W1998371279 hasConcept C142724271 @default.
- W1998371279 hasConcept C143065580 @default.
- W1998371279 hasConcept C151730666 @default.
- W1998371279 hasConcept C169760540 @default.
- W1998371279 hasConcept C181199279 @default.
- W1998371279 hasConcept C25498285 @default.
- W1998371279 hasConcept C2776925932 @default.
- W1998371279 hasConcept C2779134260 @default.
- W1998371279 hasConcept C2779343474 @default.
- W1998371279 hasConcept C2780647506 @default.
- W1998371279 hasConcept C2781307694 @default.
- W1998371279 hasConcept C2781427258 @default.
- W1998371279 hasConcept C2796436 @default.
- W1998371279 hasConcept C54355233 @default.
- W1998371279 hasConcept C55493867 @default.
- W1998371279 hasConcept C71924100 @default.
- W1998371279 hasConcept C86803240 @default.
- W1998371279 hasConcept C95444343 @default.
- W1998371279 hasConceptScore W1998371279C102230213 @default.
- W1998371279 hasConceptScore W1998371279C104317684 @default.
- W1998371279 hasConceptScore W1998371279C141035611 @default.
- W1998371279 hasConceptScore W1998371279C142724271 @default.
- W1998371279 hasConceptScore W1998371279C143065580 @default.
- W1998371279 hasConceptScore W1998371279C151730666 @default.
- W1998371279 hasConceptScore W1998371279C169760540 @default.
- W1998371279 hasConceptScore W1998371279C181199279 @default.
- W1998371279 hasConceptScore W1998371279C25498285 @default.
- W1998371279 hasConceptScore W1998371279C2776925932 @default.
- W1998371279 hasConceptScore W1998371279C2779134260 @default.
- W1998371279 hasConceptScore W1998371279C2779343474 @default.
- W1998371279 hasConceptScore W1998371279C2780647506 @default.
- W1998371279 hasConceptScore W1998371279C2781307694 @default.
- W1998371279 hasConceptScore W1998371279C2781427258 @default.
- W1998371279 hasConceptScore W1998371279C2796436 @default.
- W1998371279 hasConceptScore W1998371279C54355233 @default.
- W1998371279 hasConceptScore W1998371279C55493867 @default.
- W1998371279 hasConceptScore W1998371279C71924100 @default.
- W1998371279 hasConceptScore W1998371279C86803240 @default.
- W1998371279 hasConceptScore W1998371279C95444343 @default.
- W1998371279 hasIssue "Suppl 1" @default.
- W1998371279 hasLocation W19983712791 @default.
- W1998371279 hasOpenAccess W1998371279 @default.
- W1998371279 hasPrimaryLocation W19983712791 @default.
- W1998371279 hasRelatedWork W1964953273 @default.
- W1998371279 hasRelatedWork W2018623984 @default.
- W1998371279 hasRelatedWork W2027589670 @default.
- W1998371279 hasRelatedWork W2056730920 @default.
- W1998371279 hasRelatedWork W2058827936 @default.
- W1998371279 hasRelatedWork W2065599314 @default.
- W1998371279 hasRelatedWork W2500477896 @default.
- W1998371279 hasRelatedWork W3210985973 @default.
- W1998371279 hasRelatedWork W4296999292 @default.
- W1998371279 hasRelatedWork W2769343470 @default.
- W1998371279 hasVolume "83" @default.
- W1998371279 isParatext "false" @default.
- W1998371279 isRetracted "false" @default.
- W1998371279 magId "1998371279" @default.
- W1998371279 workType "article" @default.