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Konno, Takuya

https://orcid.org/0000-0002-2685-0025

Mayo Clinic

Mayo Clinic

Citations 24

Genetic Screening of CSF1R in Familial Ischemic Stroke (S48.008)
Rademakers, Rosa , Konno, Takuya , Miura, Takeshi , Harriott, Andrea M. , Mezaki, Naomi , Edwards, Emily, Ross, Owen A. , Meschia, James F. , Ikeuchi, Takeshi , Wszołek, Zbigniew K.

Neurology | December 7, 2023 | Volume: 90 | Issue: 15_supplement | Pages: No page available

10.1212/wnl.90.15_supplement.s48.008 Get It

Genetic risk variants for Parkinson’s disease (PD) and cognitive impairment in four clinical PD subtypes (S3.004)
Deutschländer, Angela , Soto, Alexandra I., Ossi, Maryam, Strongosky, Audrey , Uitti, Ryan J. , Gerpen, Jay Van, Konno, Takuya , Heckman, Michael G. , Gerpen, Jay A. van, Ross, Owen A. , Wszołek, Zbigniew K.

Neurology | December 7, 2023 | Volume: 90 | Issue: 15_supplement | Pages: No page available

10.1212/wnl.90.15_supplement.s3.004 Get It

Family with brain calcification and SLC20A2 variant – possible anticipation (P6.051)
Blackburn, Patrick R. , Rozen, Todd D. , Gerpen, Jay Van, Konno, Takuya , Gerpen, Jay A. van, Ross, Owen A. , Atwal, Paldeep S , Wszołek, Zbigniew K.

Neurology | December 7, 2023 | Volume: 90 | Issue: 15_supplement | Pages: No page available

10.1212/wnl.90.15_supplement.p6.051 Get It

Deep Brain Stimulation May Be Beneficial for Levodopa-Refractory Tremor in Benign Tremulous Parkinsonism (P4.335)
Wharen, Robert E. , Uitti, Ryan J. , Konno, Takuya , Ross, Owen A. , Wszołek, Zbigniew K.

Neurology | December 7, 2023 | Volume: 86 | Issue: 16_supplement | Pages: No page available

10.1212/wnl.86.16_supplement.p4.335 Get It

Proposed Diagnostic Criteria for Adult-onset Leukoencephalopathy with Axonal Spheroids and Pigmented Glia (P2.238)
Yoshida, Kunihiro , Kawarai, Toshitaka , Tada, Masayoshi, Nozaki, Hiroaki , Ikeda, Shu‐ichi , Nishizawa, Masatoyo , Konno, Takuya , Mizuno, Toshiki , Onodera, Osamu , Wszołek, Zbigniew K. , Ikeuchi, Takeshi

Neurology | December 7, 2023 | Volume: 86 | Issue: 16_supplement | Pages: No page available

10.1212/wnl.86.16_supplement.p2.238 Get It

Comparison of Clinical Features Among Parkinson Disease Subtypes (P6.014)
Ossi, Maryam, Vargas, Emily , Strongosky, Audrey , Wszolek, Zbigniew, Konno, Takuya , Heckman, Michael G. , Ross, Owen A. , Wszołek, Zbigniew K.

Neurology | December 7, 2023 | Volume: 88 | Issue: 16_supplement | Pages: No page available

10.1212/wnl.88.16_supplement.p6.014 Get It

DCTN1-related Neurodegeneration: Perry Syndrome and Beyond (P1.108)
Konno, Takuya , Ross, Owen A. , Teive, Hélio Afonso Ghizoni , Olaya, Mercedes , Dickson, Dennis W. , Wszołek, Zbigniew K. , Olaya, Mercedes

Neurology | December 7, 2023 | Volume: 88 | Issue: 16_supplement | Pages: No page available

10.1212/wnl.88.16_supplement.p1.108 Get It

Author Response: FUS regulates RAN translation through modulating the G-quadruplex structure of GGGGCC repeat RNA in C9orf72-linked ALS/FTD
Fujino, Yuzo , Ishiguro, Taro , Ozawa, Daisaku, Ito, Hayato , Taminato, Tomoya , Kawabe, Yuya , Takeuchi, Toshihide , Ikeda, Manabu , Mizusawa, Hidehiro, Wada, Keiji , Ishikawa, Kinya , Petrucelli, Leonard , Ueyama, Morio , Sugiki, Toshihiko , Murata, Asako , Ishiguro, Akira , Gendron, Tania F. , Mori, Kohji , Tokuda, Eiichi , Konno, Takuya , Koyama, Akihide , Furukawa, Yoshiaki , Fujiwara, Toshimichi , Mizuno, Toshiki , Mochizuki, Hideki , Onodera, Osamu , Nakatani, Kazuhiko , Taguchi, Hideki , Nagai, Yoshitaka

No container title available | July 18, 2023 | Volume: No volume available | Issue: No issue available | Pages: No page available

10.7554/elife.84338.3.sa4 Get It

FUS regulates RAN translation through modulating the G-quadruplex structure of GGGGCC repeat RNA in C9orf72-linked ALS/FTD
Fujino, Yuzo , Ishiguro, Taro , Ozawa, Daisaku, Ito, Hayato , Sugiki, Toshihiko , Mori, Kohji , Taminato, Tomoya , Kawabe, Yuya , Takeuchi, Toshihide , Ikeda, Manabu , Mizusawa, Hidehiro, Wada, Keiji , Ishikawa, Kinya , Petrucelli, Leonard , Ito, Hayato , Sugiki, Toshihiko , Mori, Kohji , Ueyama, Morio , Murata, Asako , Ishiguro, Akira , Gendron, Tania F. , Tokuda, Eiichi , Konno, Takuya , Koyama, Akihide , Furukawa, Yoshiaki , Fujiwara, Toshimichi , Mizuno, Toshiki , Mochizuki, Hideki , Onodera, Osamu , Nakatani, Kazuhiko , Taguchi, Hideki , Nagai, Yoshitaka

eLife | July 18, 2023 | Volume: 12 | Issue: No issue available | Pages: No page available

10.7554/elife.84338.3 Get It

Abnormal expansions of GGGGCC repeat sequence in the noncoding region of the C9orf72 gene is the most common cause of familial amyotrophic lateral sclerosis and frontotemporal dementia (C9-ALS/FTD). The expanded repeat sequence is translated into dipeptide repeat proteins (DPRs) by noncanonical repeat-associated non-AUG (RAN) translation. Since DPRs play central roles in the pathogenesis of C9-ALS/FTD, we here investigate the regulatory mechanisms of RAN translation, focusing on the effects of RNA-binding proteins (RBPs) targeting GGGGCC repeat RNAs. Using C9-ALS/FTD model flies, we demonstrated that the ALS/FTD-linked RBP FUS suppresses RAN translation and neurodegeneration in an RNA-binding activity-dependent manner. Moreover, we found that FUS directly binds to and modulates the G-quadruplex structure of GGGGCC repeat RNA as an RNA chaperone, resulting in the suppression of RAN translation in vitro. These results reveal a previously unrecognized regulatory mechanism of RAN translation by G-quadruplex-targeting RBPs, providing therapeutic insights for C9-ALS/FTD and other repeat expansion diseases.
FUS regulates RAN translation through modulating the G-quadruplex structure of GGGGCC repeat RNA in C9orf72-linked ALS/FTD
Fujino, Yuzo , Ishiguro, Taro , Ozawa, Daisaku, Ito, Hayato , Sugiki, Toshihiko , Mori, Kohji , Taminato, Tomoya , Kawabe, Yuya , Takeuchi, Toshihide , Ikeda, Manabu , Mizusawa, Hidehiro, Wada, Keiji , Ishikawa, Kinya , Petrucelli, Leonard , Sugiki, Toshihiko , Mori, Kohji , Ueyama, Morio , Murata, Asako , Ishiguro, Akira , Gendron, Tania F. , Tokuda, Eiichi , Konno, Takuya , Koyama, Akihide , Furukawa, Yoshiaki , Fujiwara, Toshimichi , Mizuno, Toshiki , Mochizuki, Hideki , Onodera, Osamu , Nakatani, Kazuhiko , Taguchi, Hideki , Nagai, Yoshitaka

No container title available | June 20, 2023 | Volume: No volume available | Issue: No issue available | Pages: No page available

10.7554/elife.84338.2 Get It

Abstract Abnormal expansions of GGGGCC repeat sequence in the noncoding region of the C9orf72 gene is the most common cause of familial amyotrophic lateral sclerosis and frontotemporal dementia (C9-ALS/FTD). The expanded repeat sequence is translated into dipeptide repeat proteins (DPRs) by noncanonical repeat-associated non-AUG (RAN) translation. Since DPRs play central roles in the pathogenesis of C9-ALS/FTD, we here investigate the regulatory mechanisms of RAN translation, focusing on the effects of RNA-binding proteins (RBPs) targeting GGGGCC repeat RNAs. Using C9-ALS/FTD model flies, we demonstrated that the ALS/FTD-linked RBP FUS suppresses RAN translation and neurodegeneration in an RNA-binding activity-dependent manner. Moreover, we found that FUS directly binds to and modulates the G-quadruplex structure of GGGGCC repeat RNA as an RNA chaperone, resulting in the suppression of RAN translation in vitro. These results reveal a previously unrecognized regulatory mechanism of RAN translation by G-quadruplex-targeting RBPs, providing therapeutic insights for C9-ALS/FTD and other repeat expansion diseases.