Rinsho Shinkeigaku (Clinical Neurology)

Special article by the winner of Japanese Society of Neurology

Identification and significance of a novel degradation system for polyglutamine aggregates

Atsushi Iwata, M.D., Ph.D.1)

1)University of Tokyo Hospital, Department of Neurology

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.
Full Text of this Article in Japanese PDF (3079K)

(CLINICA NEUROL, 53: 1|8, 2013)
key words: polyglutamine, autophagy, aggresomes, ubiquitin proteasome, HDAC6

(Received: 9-Oct-12)