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研究報告:2018年

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1.      Tohnai G, Nakamura R, Sone J, Nakatochi M, Yokoi D, Katsuno M, Watanabe H, Ito M, Li Y, Izumi Y, Morita M, Taniguchi A, Kano O, Oda M, Kuwabara S, Abe K, Aiba I, Okamoto K, Mizoguchi K, Hasegawa K, Aoki M, Hattori N, Onodera O, Naruse H, Mitsui J, Takahashi Y, Goto J, Ishiura H, Morishita S, Yoshimura J, Doi K, Tsuji S, Nakashima K, Kaji R, Atsuta N, Sobue G; Japanese Consortium for Amyotrophic Lateral Sclerosis Research (JaCALS): Frequency and characteristics of the TBK1 gene variants in Japanese patients with sporadic amyotrophic lateral sclerosis. Neurobiol Aging. 64: 158.e15–158.e19, 2018

2.      Yoneyama N, Watanabe H, Kawabata K, Bagarinao E, Hara K, Tsuboi T, Tanaka Y, Ohdake R, Imai K, Masuda M, Hattori T, Ito M, Atsuta N, Nakamura T, Hirayama M, Maesawa S, Katsuno M, Sobue G: Severe hyposmia and aberrant functional connectivity in cognitively normal Parkinson’s disease. PLoS One. 13: e0190072, 2018

3.      Ninomiya H, Ohgami N, Oshino R, Kato M, Ohgami K, Li X, Shen D, Iida M, Yajima I, Angelidis CE, Adachi H, Katsuno M, Sobue G, Kato M: Increased expression level of Hsp70 in the inner ears of mice by exposure to low frequency noise. Hear Res. 363: 49–54, 2018

4.      Koike H, Nishi R, Ikeda S, Kawagashira Y, Iijima M, Atsuta N, Nakamura T, Hirayama M, Ogata H, Yamasaki R, Kira JI, Katsuno M, Sobue G: Restoration of a Conduction Block after the Long-term Treatment of CIDP with Anti-neurofascin 155 Antibodies: Follow-up of a Case over 23 Years. Intern Med. 57: 2061–2066, 2018

5.      Tanikawa C, Ueda K, Suzuki A, Iida A, Nakamura R, Atsuta N, Tohnai G, Sobue G, Saichi N, Momozawa Y, Kamatani Y, Kubo M, Yamamoto K, Nakamura Y, Matsuda K: Citrullination of RGG Motifs in FET Proteins by PAD4 Regulates Protein Aggregation and ALS Susceptibility. Cell Rep. 22: 1473–1483, 2018

6.      Sekijima Y, Yazaki M, Ueda M, Koike H, Yamada M, Ando Y: First nationwide survey on systemic wild-type ATTR amyloidosis in Japan. Amyloid. 25: 8–10, 2018

7.      Hijikata Y, Katsuno M, Suzuki K, Hashizume A, Araki A,Yamada S, Inagaki T, Ito D, Hirakawa A, Kinoshita F, Gosho M, Sobue G: Treatment with Creatine Monohydrate in Spinal and Bulbar Muscular Atrophy: Protocol for a Randomized, Double-Blind, Placebo-Controlled Trial. JMIR Res Protoc. 7: e69, 2018

8.      Hijikata Y, Hashizume A,Yamada S, Inagaki T, Ito D, Hirakawa A, Suzuki K, Atsuta N,Tsuboi T, Hattori M, Hori A, Banno H, Sobue G, Katsuno M: Biomarker-based analysis of preclinical progression in spinal and bulbar muscular atrophy. Neurology. 90: e1501–e1509, 2018

9.      Kawabata K, Watanabe H, Hara K, Bagarinao E, Yoneyama N, Ogura A, Imai K, Masuda M, Yokoi T, Ohdake R, Tanaka Y,Tsuboi T, Nakamura T, Hirayama M, Ito M, Atsuta N, Maesawa S, Naganawa S, Katsuno M, Sobue G: Distinct manifestation of cognitive deficits associate with different resting-state network disruptions in non-demented patients with Parkinson’s disease. J Neurol. 265: 688–700, 2018

10.    Koike H, Katsuno M, Sobue G: Deciphering mechanism and spectrum of chronic inflammatory demyelinating polyneuropathy by morphology. Clin Exp Neuroimmunol. 9: 35–46, 2018

11.    Ikumi K, Tsuboi T, Atsuta N, Takeuchi K, Koike H, Katsuno M: Long-standing overt ventriculomegaly without aqueductal stenosis: A case report. Neurol Clin Neurosci. 6: 42–44, 2018

12.    Tsuboi T, Watanabe H, Funasaka K, Takebayashi M, Miyata K and Katsuno M: Peritonitis after percutaneous endoscopic gastrojejunostomy for levodopa-carbidopa intestinal gel treatment despite concomitant use of gastropexy. Neurol Clin Neurosci. 6: 64–66, 2018

13.    Bagarinao E, Watanabe H, Maesawa S, et al.: An unbiased data-driven age-related structural brain parcellation for the identification of intrinsic brain volume changes over the adult lifespan. Neuroimage. 169: 134–144, 2018

14.    Sakamoto Y, Okamoto S, Maesawa S, Bagarinao E, Araki Y, Izumi T, Watanabe H, Sobue G, Wakabayashi T: Default Mode Network Changes in Moyamoya Disease Before and After Bypass Surgery: Preliminary Report. World Neurosurg. 112: e652–e661, 2018

15.    Koike H, Nishi R, Ikeda S, Kawagashira Y, Iijima M, Katsuno M, Sobue G: Ultrastructural mechanisms of macrophage-induced demyelination in CIDP. Neurology. 91: 1051–1060, 2018

16.    Masuda M, Watanabe H, Tanaka Y, Ohdake R, Ogura A, Yokoi T, Imai K, Kawabata K, Riku Y, Hara K, Nakamura R, Atsuta N, Katsuno M, Sobue G: Age-related impairment in Addenbrooke’s cognitive examination revised scores in patients with amyotrophic lateral sclerosis. Amyotroph Lateral Scler Frontotemporal Degener. 19: 578–584, 2018

17.    Yokoi T, Watanabe H, Yamaguchi H, Bagarinao E, Masuda M, Imai K, Ogura A, Ohdake R, Kawabata K, Hara K, Riku Y, Ishigaki S, Katsuno M , Miyao S, Kato K, Naganawa S, HaradaR, Okamura N, Yanai K, Yoshida M, Sobue G: Involvement of the Precuneus/Posterior Cingulate Cortex Is Significant for the Development of Alzheimer’s Disease: A PET (THK5351, PiB) and Resting fMRI Study. Front Aging Neurosci. 10: 304, 2018

18.    Hashizume A, Katsuno M: IGF-1 for spinal and bulbar muscular atrophy: hope and challenges. Lancet Neurol. 17: 1026–1027, 2018

19.    Koike H, Nishi R, Ikeda S, Kawagashira Y, Iijima M, Sakurai T, Shimohata T, Katsuno M, Sobue G: The morphology of amyloid fibrils and their impact on tissue damage in hereditary transthyretin amyloidosis: An ultrastructural study. J Neurol Sci. 394: 99–106, 2018

20.    Katsuno M, Sahashi K, Iguchi Y, Hashizume A: Preclinical progression of neurodegenerative diseases. Nagoya J Med Sci. 80: 289–298, 2018

21.    Yamada S, Hashizume A, Hijikata Y, Inagaki T, Ito D, Kinoshita F, Nakatochi M, Kobayashi Y, Hirakawa A, Nakamura T, Katsuno M: Study protocol for the MEXiletine hydrochloride administration trial: a placebocontrolled, randomised, double-blind, multicentre, crossover study of its efficacy and safety in spinal and bulbar muscular atrophy (MEXPRESS). BMJ Open. 8: e023041, 2018

22.    Watanabe H, Riku Y, Hara K, Kawabata K, Nakamura T, Ito M, Hirayama M, Yoshida M, Katsuno M, Sobue G: Clinical and Imaging Features of Multiple System Atrophy: Challenges for an Early and Clinically Definitive Diagnosis. J Mov Disord. 11: 107–120, 2018

23.    Koike H, Nakamura T, Nishi R, Ikeda S, Kawagashira Y, Iijima M, Katsuno M, Sobue G: Widespread Cardiac and Vasomotor Autonomic Dysfunction in Non-Val30MetHereditary Transthyretin Amyloidosis. Intern Med. 57: 3365–3370, 2018

24.    Hara K, Watanabe H, Bagarinao E, Kawabata K, Yoneyama N, Ohdake R, Imai K, Masuda M, Yokoi T, Ogura A, Tsuboi T, Ito M, Atsuta N, Niwa H, Taoka T, Maesawa S, Naganawa S, Katsuno M, Sobue G: Corpus callosal involvement is correlated with cognitive impairment in multiple system atrophy. J Neurol. 265: 2079–2087, 2018

25.    Xu Y, Halievski K, Katsuno M, Adachi H, Sobue G, Breedlove SM, Jordan CL: Pre-clinical symptoms of SBMA may not be androgen-dependent: implications from two SBMA mouse models. Hum Mol Genet. 27: 2425–2442, 2018

26.    Koike H, Yasuda T, Nishi R, Ikeda S, Kawagashira Y, Iijima M, Sobue G, Katsuno M: Systemic angiopathy and axonopathy in hereditary transthyretin amyloidosis with Ala97Gly (p. Ala117Gly) mutation: a post-mortem analysis. Amyloid. 25: 141–142, 2018

27.    Ando T, Goto Y, Mano K, Nomura F, Kurashige M, Ito M, Mimuro M, Iwasaki Y, Katsuno M, Yoshida M:
Paraneoplastic autoimmune encephalitis associated with pleomorphic lung carcinoma: An autopsy case report. Neuropathology. 2018 May 20

28.    Hijikata Y, Hashizume A, Yamada S, Inagaki T, Ito D, Hirakawa A, Suzuki K, Atsuta N, Tsuboi T, Hattori M, Hori A, Banno H, Sobue G, Katsuno M: Biomarker-based analysis of preclinical progression in spinal and bulbar muscular atrophy. Neurology. 90: e1501–e1509, 2018

29.    Ninomiya H, Ohgami N, Oshino R, Kato M, Ohgami K, Li X, Shen D, Iida M, Yajima I, Angelidis CE, Adachi H, Katsuno M, Sobue G, Kato M: Increased expression level of Hsp70 in the inner ears of mice by exposure to low frequency noise. Hear Res. 363: 49–54, 2018

30.    Koike H, Nishi R, Ikeda S, Kawagashira Y, Iijima M, Atsuta N, Nakamura T, Hirayama M, Ogata H, Yamasaki R, Kira JI, Katsuno M, Sobue G: Restoration of a Conduction Block after the Long-term Treatment of CIDP with Anti-neurofascin 155 Antibodies: Follow-up of a Case over 23 Years. Intern Med. 57: 2061–2066, 2018

31.    Tohnai G, Nakamura R, Sone J, Nakatochi M, Yokoi D, Katsuno M, Watanabe H, Ito M, Li Y, Izumi Y, Morita M, Taniguchi A, Kano O, Oda M, Kuwabara S, Abe K, Aiba I, Okamoto K, Mizoguchi K, Hasegawa K, Aoki M, Hattori N, Onodera O, Naruse H, Mitsui J, Takahashi Y, Goto J, Ishiura H, Morishita S, Yoshimura J, Doi K, Tsuji S, Nakashima K, Kaji R, Atsuta N, Sobue G ; Japanese Consortium for Amyotrophic Lateral Sclerosis Research (JaCALS): Frequency and characteristics of the TBK1 gene variants in Japanese patients with sporadic amyotrophic lateral sclerosis. Neurobiol Aging. 64: 158.e15–158.e19, 2018

32.    Kawabata K, Watanabe H, Hara K, Bagarinao E, Yoneyama N, Ogura A, Imai K, Masuda M, Yokoi T, Ohdake R, Tanaka Y, Tsuboi T, Nakamura T, Hirayama M, Ito M, Atsuta N, Maesawa S, Naganawa S, Katsuno M, Sobue G: Distinct manifestation of cognitive deficits associate with different resting-state network disruptions in non-demented patients with Parkinson’s disease. J Neurol. 265: 688–700, 2018

33.    Endo K, Ishigaki S, Masamizu Y, Fujioka Y, Watakabe A, Yamamori T, Hatanaka N, Nambu A, Okado H, Katsuno M, Watanabe H, Matsuzaki M, Sobue G: Silencing of FUS in the common marmoset (Callithrix jacchus) brain via stereotaxic injection of an adeno-associated virus encoding shRNA. Neurosci Res. 130: 56–64, 2018

34.    Suzuki A, Ito M, Hamaguchi T, Mori H, Takeda Y, Baba R, Watanabe T, Kurokawa K, Asakawa S, Hirayama M, Ohno K: Quantification of hydrogen production by intestinal bacteria that are specifically dysregulated in Parkinson’s disease. PLoS One. 13: e0208313, 2018

35.    Abe K, Hirayama M, Ohno K, Shimamura T: A latent allocation model for the analysis of microbial composition and disease. BMC Bioinformatics. 19: 519, 2018

36.    Misawa S, Kuwabara S, Sato Y, Yamaguchi N, Nagashima K, Katayama K, Sekiguchi Y, Iwai Y, Amino H, Suichi T, Yokota T, Nishida Y, Kanouchi T, Kohara N, Kawamoto M, Ishii J, Kuwahara M, Suzuki H, Hirata K, Kokubun N, Masuda R, Kaneko J, Yabe I, Sasaki H, Kaida KI, Takazaki H, Suzuki N, Suzuki S, Nodera H, Matsui N, Tsuji S, Koike H, Yamasaki R, Kusunoki S; Japanese Eculizumab Trial for GBS (JET-GBS) Study Group: Safety and efficacy of eculizumab in Guillain-Barré syndrome: a multicentre, doubleblind, randomised phase 2 trial. Lancet Neurol. 17: 519–529, 2018

37.    Watanabe E, Fujita T, Shimono M, Koike H, Yasumoto S, Hirose S: Recurrent autonomic and sensory neuropathy in a patient with anti-ganglionic acetylcholine receptor antibodies. eNeurologicalSci. 12: 36–38, 2018

38.    Sekijima Y, Yazaki M, Ueda M, Koike H, Yamada M, Ando Y: First nationwide survey on systemic wild-type ATTR amyloidosis in Japan. Amyloid. 25: 8–10, 2018

39.    Fujimori K, Ishikawa M, Otomo A, Atsuta N, Nakamura R, Akiyama T, Hadano S, Aoki M, Saya H, Sobue G, Okano H: Modeling sporadic ALS in iPSC-derived motor neurons identifies a potential therapeutic agen.t Nat Med. 24: 1579–1589, 2018

40.    Sobue G, Ishigaki S, Watanabe H: Pathogenesis of Frontotemporal Lobar Degeneration: Insights From Loss of Function Theory and Early Involvement of the Caudate Nucleus. Front Neurosci. 12: 473, 2018

41.    Ishigaki S, Sobue G: Importance of Functional Loss of FUS in FTLD/ALS. Front Mol Biosci. 5: 44, 2018

42.    Oki R, Izumi Y, Nodera H, Sato Y, Nokihara H, Kanai K, Sonoo M, Urushitani M, Nishinaka K, Atsuta N, Kohara N, Shimizu T, Kikuchi H, Oda M, Ikeda K, Nagai M, Komai K, Kojima Y, Kuzume D, Isose S, Shimohama S, Abe K, Ito H, Noda K, Ishihara T, Morita M, Shimohata T, Teramukai S, Kagimura T, Noma K, Yanagawa H, Kuwabara S, Kaji R; JETALS: The Japanese Early-Stage Trial of High-Dose Methylcobalamin for Amyotrophic Lateral Sclerosis (JETALS): Protocol for a Randomized Controlled Trial. JMIR Res Protoc. 7: e12046, 2018
Sahashi K, Katsuno M: Pathogenesis of Polyglutamine Diseases eLS. John Wiley & Sons, Ltd: Chichester. Sinha R, Kim YJ, Nomakuchi T, Sahashi K, Hua Y, Rigo F, Bennett CF, Krainer AR: Antisense oligonucleotides correct the familial dysautonomia splicing defect in IKBKAP transgenic mice. Nucleic Acids Res. 46: 4833–4844, 2018

43.    Kuwabara S, Misawa S, Mori M, Iwai Y, Ochi K, Suzuki H, Nodera H, Tamaoka A, Iijima M, Toda T, Yoshikawa H, Kanda T, Sakamoto K, Kusunoki S, Sobue G, Kaji R; Glovenin-I MMN Study Group: Intravenous immunoglobulin for maintenance treatment of multifocal motor neuropathy: A multi-center, open-label, 52-week phase 3 trial. J Peripher Nerv Syst. 23: 115–119, 2018

44.    Iijima M: Animal models of chronic inflammatory demyelinating polyneuropathy. Clin Exp Neuroimmunol. 9: 101–109, 2018

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