@article{oai:repo.qst.go.jp:00047794,
 author = {Ishigaki, Shinsuke and Fujioka, Yusuke and Okada, Yohei and Riku, Yuichi and Udagawa, Tsuyoshi and Honda, Daiyu and Yokoi, Satoshi and Endo, Kuniyuki and Ikenaka, Kensuke and Takagi, Shinnosuke and Iguchi, Yohei and Sahara, Naruhiko and Takashima, Akihiko and Okano, Hideyuki and Yoshida, Mari and Warita, Hitoshi and Aoki, Masashi and Watanabe, Hirohisa and Okado, Haruo and Katsuno, Masahisa and Sobue, Gen and 佐原 成彦},
 issue = {5},
 journal = {Cell reports},
 month = {Jan},
 note = {Fused in sarcoma (FUS) and splicing factor, prolineand
glutamine-rich (SFPQ) are RNA binding proteins
that regulate RNA metabolism. We found that alternative
splicing of the Mapt gene at exon 10, which
generates 4-repeat tau (4R-T) and 3-repeat tau
(3R-T), is regulated by interactions between FUS
and SFPQ in the nuclei of neurons. Hippocampusspecific
FUS- or SFPQ-knockdown mice exhibit
frontotemporal lobar degeneration (FTLD)-like behaviors,
reduced adult neurogenesis, accumulation
of phosphorylated tau, and hippocampal atrophy
with neuronal loss through an increased 4R-T/3R-T
ratio. Normalization of this increased ratio by 4R-Tspecific
silencing results in recovery of the normal
phenotype. These findings suggest a biological link
among FUS/SFPQ, tau isoform alteration, and
phenotypic expression, which may function in the
early pathomechanism of FTLD.},
 pages = {1118--1131},
 title = {Altered tau isoform ratio caused by loss of FUS and SFPQ function leads to FTLD-like phenotypes},
 volume = {18},
 year = {2017}
}