title: [Identification and significance of a novel degradation system for polyglutamine aggregates].
abstract: In neurodegeneration, accumulation of aggregated protein results in the formation of inclusion bodies, which is a pathological hallmark of the diseases. There are various forms of aggregates; from oligomers to filaments and to inclusion bodies, of which small species such as oligomers are considered to be more toxic compared to tightly packed inclusion bodies. In vitro experiments show that artificial inclusion bodies called aggresomes are generated with motor proteins and microtubules, which represents cellular effort to manage aggregated proteins. We showed that the aggresomes are formed in order to concentrate autophagy components and aggregates in order to give specificity to autophagy against aggregated proteins, thus facilitating their selective degradation.
authors: University of Tokyo Hospital, Department of Neurology.
authors: Atsushi
authors: A
authors: Iwata
mesh_terms: D000371:Agglutination; D001343:Autophagy / Q000502:physiology; D001921:Brain / Q000378:metabolism*; D000073864:Histone Deacetylase 6; D006655:Histone Deacetylases / Q000378:metabolism; D002479:Inclusion Bodies / Q000378:metabolism; D008247:Lysosomes / Q000502:physiology; D008870:Microtubules / Q000502:physiology; D020409:Molecular Motor Proteins / Q000502:physiology; D019636:Neurodegenerative Diseases / Q000209:etiology* / Q000378:metabolism*; D010455:Peptides / Q000378:metabolism*; D046988:Proteasome Endopeptidase Complex / Q000502:physiology; D011485:Protein Binding; D025801:Ubiquitin / Q000502:physiology; D057149:Ubiquitinated Proteins / Q000378:metabolism*
chemical_list: D020409:Molecular Motor Proteins; D010455:Peptides; D025801:Ubiquitin; D057149:Ubiquitinated Proteins; C097188:polyglutamine; D046988:Proteasome Endopeptidase Complex; C433811:HDAC6 protein, human; D000073864:Histone Deacetylase 6; D006655:Histone Deacetylases