Reduction in PA28αβ activation in HD mouse brain correlates to increased mHTT aggregation in cell models

Huntington's disease is an autosomal dominant heritable disorder caused by an expanded CAG trinucleotide repeat at the N-terminus of the Huntingtin (HTT) gene. Lowering the levels of soluble mutant HTT protein prior to aggregation through increased degradation by the proteasome would be a thera...

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Veröffentlicht in:	PloS one 2022-12, Vol.17 (12), p.e0278130-e0278130
Hauptverfasser:	Geijtenbeek, Karlijne W, Janzen, Jolien, Bury, Aleksandra E, Sanz-Sanz, Alicia, Hoebe, Ron A, Bondulich, Marie K, Bates, Gillian P, Reits, Eric A J, Schipper-Krom, Sabine
Format:	Artikel
Sprache:	eng
Schlagworte:	Agglomeration Animals Autophagy Biology and Life Sciences Brain - metabolism Brain stem Brain Stem - metabolism Cell culture Cerebellum Cerebellum - metabolism Cloning Cytoplasm Degradation Disease Models, Animal Glycerol Hereditary diseases Huntingtin Huntingtin Protein - genetics Huntingtin Protein - metabolism Huntington Disease - metabolism Huntington's disease Huntingtons disease Labeling Medicine and Health Sciences Mice Mutants N-Terminus Neostriatum Peptides Polyglutamine Proteasome Endopeptidase Complex - genetics Proteasome Endopeptidase Complex - metabolism Proteasomes Proteins Proteostasis Research and Analysis Methods Trinucleotide repeats
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