Elavl3 is essential for the maintenance of Purkinje neuron axons

Yuki Ogawa, Kyoko Kakumoto, Tetsu Yoshida, Ken ichiro Kuwako, Taisuke Miyazaki, Junji Yamaguchi, Ayumu Konno, Junichi Hata, Yasuo Uchiyama, Hirokazu Hirai, Masahiko Watanabe, Robert B. Darnell, Hideyuki Okano, Hirotaka James Okano

研究成果: Article査読

22 被引用数 (Scopus)

抄録

Neuronal Elav-like (nElavl or neuronal Hu) proteins are RNA-binding proteins that regulate RNA stability and alternative splicing, which are associated with axonal and synaptic structures. nElavl proteins promote the differentiation and maturation of neurons via their regulation of RNA. The functions of nElavl in mature neurons are not fully understood, although Elavl3 is highly expressed in the adult brain. Furthermore, possible associations between nElavl genes and several neurodegenerative diseases have been reported. We investigated the relationship between nElavl functions and neuronal degeneration using Elavl3−/− mice. Elavl3−/− mice exhibited slowly progressive motor deficits leading to severe cerebellar ataxia, and axons of Elavl3−/− Purkinje cells were swollen (spheroid formation), followed by the disruption of synaptic formation of axonal terminals. Deficit in axonal transport and abnormalities in neuronal polarity was observed in Elavl3−/− Purkinje cells. These results suggest that nElavl proteins are crucial for the maintenance of axonal homeostasis in mature neurons. Moreover, Elavl3−/− mice are unique animal models that constantly develop slowly progressive axonal degeneration. Therefore, studies of Elavl3−/− mice will provide new insight regarding axonal degenerative processes.

本文言語English
論文番号2722
ジャーナルScientific reports
8
1
DOI
出版ステータスPublished - 2018 12月 1

ASJC Scopus subject areas

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