1. Sato, K., Otomo, A., Ueda, M. T., Hiratsuka, Y., Suzuki-Utsunomiya, K., Sugiyama, J., Murakoshi, S., Mitsui, S., Ono, S., Nakagawa, S., Shang, H. -F., and Hadano, S. (2018) Altered oligomeric states in pathogenic ALS2 variants associated with juvenile motor neuron diseases cause loss of ALS2-mediated endosomal function. J. Biol. Chem. 293 (44), 17135-17153.
  2. Fujimori, K., Ishikawa, M., Otomo, A., Atsuta, N., Nakamura, R., Akiyama, T., Hadano, S., Aoki, M., Saya, H., Sobue, G., and Okano, H. (2018) Modeling sporadic ALS in iPSC-derived motor neurons identifies a potential therapeutic agent. Nat. Med. 24 (10), 1579-1589.
  3. Mitsui, S., Otomo, A., Nozaki, M., Ono, S., Sato, K., Shirakawa, R., Adachi, H., Aoki, M., Sobue, G., Shang, H. -F., and Hadano, S. (2018) Systemic overexpression of SQSTM1/p62 accelerates disease onset in a SOD1H46R-expressing ALS mouse model. Mol. Brain 11 (1), 30.
  4. Imaizumi, K., Fujimori, K., Ishii, S., Otomo, A., Hosoi, Y., Miyajima, H., Warita, H., Aoki, M., Hadano, S., Akamatsu, W., and Okano, H. (2018) Rostrocaudal areal patterning of human PSC-derived cortical neurons by FGF8 signaling. eNeuro 5 (2), e0368-17.2018 1–9.


  1. Kanno, T., Yasutake, K., Tanaka, K., Hadano, S., and Ikeda, J. -E. (2017) A novel function of N-linked glycoproteins, alpha-2-HS-glycoprotein and hemopexin: Implications for small molecule compound-mediated neuroprotection. PLoS ONE 12 (10), e0186227.
  2. 井上陽子、大友麻子、高橋千果、森屋宏美、大貫優子、谷口泰史、和泉俊一郎、秦野伸二(2017)高大連携による高校生のための分子生物学実験の実践-「遺伝子」のより深い理解を求めて-. 生物教育 58 (3), 98-113.
  3. Zhou, Q. Q., Chen, Y. P., Wei, Q. -Q., Cao, B., Wu, Y., Zhao, B., Ou, R. W., Yang, J., Chen, X. P., Hadano, S., and Shang, H. -F. (2017) Mutation screening of the CHCHD10 gene in Chinese patients with amyotrophic lateral sclerosis. Mol. Neurobiol. 54 (5), 3189-3194.
  4. Moriya, Y., Nagata, E., Fujii, N., Satoh, T., Ogawa, H., Hadano, S., and Takizawa, S. (2017) Inositol hexakisphosphate kinase 2 is a pre-symptomatic biomarker for amyotrophic lateral sclerosis. Tokai J. Exp. Clin. Med. 42 (1), 13-18.


  1. Chen, Y. P., Wei, Q. Q., Chen, X. P., Li, C. Y., Cao, B., Ou, R. W., Hadano, S., and Shang, H. -F. (2016) Aberration of miRNAs expression in leukocytes from sporadic amyotrophic lateral sclerosis. Front. Mol. Neurosci. 9, Article 69, 17 August 2016, doi: 10.3389/fnmol.2016.00069.
  2. Hadano, S., Mitsui, S., Pan, L., Otomo, A., Kubo, M., Sato, K., Ono, S., Onodera, W., Abe, K., Chen, X. P., Koike, M., Uchiyama, Y., Aoki, M., Warabi, E., Yamamoto, M., Ishii, T., Yanagawa, T., Shang, H. -F., and Yoshii, F. (2016) Functional links between SQSTM1 and ALS2 in the pathogenesis of ALS: cumulative impact on the protection against mutant SOD1-mediated motor dysfunction in mice. Hum. Mol. Genet. 25 (15), 3321-3340.
  3. Klionsky, D. J. et al , (2016) Guidelines for the use and interpretation of assays for monitoring autophagy (3rd edition). Autophagy 12 (1), 1-222.
  4. Fujitani, K., Otomo, A., Wada, M., Takamatsu, N., and Ito, M. (2016) Sexually dimorphic expression of Dmrt1 and γH2AX in germ stem cells during gonadal development in Xenopus laevis. FEBS Open Bio 6 (4), 276-284.


  1. Yang, Y., Tang, L., Zhang, N., Pan, L., Hadano, S., and Fan, D. -S. (2015) Six SQSTM1 mutations in a Chinese amyotrophic lateral sclerosis cohort. Amyotroph. Lateral Scler. Frontotempo. Degen. 16 (5-6), 378-384.


  1. Chen, Y. P., Zheng, Z. Z., Chen, X. P., Huang, R., Yang, Y., Yuan, L. X., Pan, L., Hadano, S., and Shang, H. -F. (2014) SQSTM1 mutations in Han Chinese populations with sporadic amyotrophic lateral sclerosis. Neurobiol. Aging 35 (3), 726.e7-726.e9.
  2. 秦野伸二(2014)動物実験の基本的考え方と関連法規等について. 日本香粧品学会誌 38 (4)、250-257.
  3. Suyama, K., Watanabe, M., Sakabe, K., Otomo, A., Okada, Y., Terayama, H., Imai, T., and Mochida, J. (2014) GRP78 suppresses lipid peroxidation and promotes cellular antioxidant levels in glial cells following hydrogen peroxide exposure. PLoS ONE. 9 (1), e86951.


  1. 大友麻子、白川健太郎、宮嶋裕明、秦野伸二(2013)ALS2/Alsin、アクチュアル脳・神経疾患の臨床「すべてがわかるALS・運動ニューロン疾患」IV. ALSの病態関連遺伝子と遺伝子変異、辻省次、祖父江元編、中山書店、東京、pp157-165.


  1. Kanno, T., Tanaka, K., Yanagisawa, Y., Yasutake, K., Hadano, S., Yoshii, F., Hirayama, N., and Ikeda, J. -E. (2012) A novel small-molecule, N-(4-(2-pyridyl)(1,3-thiazol-2-yl))-2-(2,4,6-trimethylphenoxy)acetamide, selectively protects against oxidative stress-induced cell death by activating the Nrf2-ARE pathway: Therapeutic implications for ALS. Free Radical Biol. Med. 53 (11), 2028-2042.
  2. Pan, L., Yoshii, Y., Otomo, A., Ogawa, H., Iwasaki, Y., Shang, H. -F., and Hadano, S. (2012) Different human copper-zinc superoxide dismutase mutants, SOD1G93A and SOD1H46R, exert distinct harmful effects on gross phenotype in mice. PLoS ONE 7 (3), e33409.
  3. Otomo, A., Pan, L., and Hadano, S. (2012) Dysregulation of the autophagy-endolysosomal system in amyotrophic lateral sclerosis and related motor neuron diseases. Neurol. Res. Int. 2012, Article ID 498428, doi:10.1155/2012/498428.


  1. Tanaka, K., Kanno, T., Yanagisawa, Y., Yasutake, K., Hadano, S., Yoshii, F., and Ikeda, J. -E. (2011) Bromocriptine methylate suppresses glial inflammation and moderates disease progression in a mouse model of amyotrophic lateral sclerosis. Exp. Neurol. 232 (1), 41-52.
  2. Yoshii, Y., Otomo, A., Pan, L., Ohtsuka, M., and Hadano, S. (2011) Loss of glial fibrillary acidic protein marginally accelerates disease progression in a SOD1H46R transgenic mouse model of ALS. Neurosci. Res. 70 (3), 321-329.
  3. Otomo, A., Kunita, R., Suzuki-Utsunomiya, K., Ikeda, J. -E., and Hadano, S. (2011) Defective relocalization of ALS2/alsin missense mutants to Rac1-induced macropinosomes accounts for loss of their cellular function and leads to disturbed amphisome formation. FEBS Lett. 585 (5), 730-736.


  1. Hadano, S., Yoshii, Y., Otomo, A., Kunita, R., Suzuki-Utsunomiya, K., Pan, L., Kakuta, S., Iwasaki, Y., Iwakura, Y., and Ikeda, J. -E. (2010) Genetic background and gender effects on gross phenotypes in congenic lines of ALS2/alsin-deficient mice. Neurosci. Res. 68 (2), 131-136.
  2. Hadano, S., Otomo, A., Kunita, R., Suzuki-Utsunomiya, K., Akatsuka, A., Koike, M., Aoki, M., Uchiyama, Y., Itoyama, Y., and Ikeda, J. -E. (2010) Loss of ALS2/alsin exacerbates motor dysfunction in a SOD1H46R-expressing mouse ALS model by disturbing endolysosomal trafficking. PLoS ONE 5 (3), e9805.
  3. Yoshii, Y., Hadano, S., Otomo, A., Kawabe, K., Ikeda, K., and Iwasaki, Y. (2010) Lower serum lipid levels are related to respiratory impairment in patients with ALS. Neurology 74 (24), 2027; author reply 2027-2028.


  1. Yoshii, Y., Hadano, S., Otomo, A., Suzuki, K., Ikeda, K., Ikeda, J. -E., and Iwasaki, Y. (2009) Natural history of young-adult amyotrophic lateral sclerosis. Neurology 73 (8), 648-649; author reply 649-650.


  1. Tanaka, K., Okada, Y., Kanno, T., Otomo, A., Yanagisawa, Y., Shouguchi-Miyata, J., Suga, E., Kohiki, E., Onoe, K., Osuga, H., Aoki, M., Hadano, S., Itoyama, Y., and Ikeda, J. -E. (2008) A dopamine receptor antagonist L-745,870 suppresses microglia activation in spinal cord and mitigates the progression in ALS model mice. Exp. Neurol. 211 (2), 378-386.
  2. Otomo, A., Kunita, R., Suzuki-Utsunomiya, K., Mizumura, H., Onoe, K., Osuga, H., Hadano, S., and Ikeda, J. -E. (2008) ALS2/alsin deficiency in neurons leads to mild defects in macropinocytosis and axonal growth. Biochem. Biophys. Res. Commn. 370 (1), 87-92.
  3. 秦野伸二(2008)ALS2遺伝子変異に起因する運動ニューロン疾患. Clinical Neuroscience 26 (3)、323-324.


  1. Kunita, R., Otomo, A., Mizumura, H., Suzuki-Utsunomiya, K., Hadano, S., and Ikeda, J. -E. (2007) The Rab5 activator ALS2/alsin acts as a novel Rac1 effector through Rac1-activated endocytosis. J. Biol. Chem. 282 (22), 16599-16611.
  2. Suzuki-Utsunomiya, K., Hadano, S., Otomo, A., Kunita, R., Mizumura, H., Osuga, H., and Ikeda, J. -E. (2007) ALS2CL, a novel ALS2-interactor, modulates ALS2-mediated endosome dynamics. Biochem. Biophys. Res. Commn. 354 (2), 491-497.
  3. Hadano, S., Kunita, R., Otomo, A., Suzuki-Utsunomiya, K., and Ikeda, J. -E. (2007) Molecular and cellular function of ALS2/alsin: Implication of membrane dynamics in neuronal development and degeneration. Neurochem. Int. 51 (2-4), 74-84.


  1. Hadano, S., Benn, S. C., Kakuta, S., Otomo, A., Sudo, K., Kunita, R., Suzuki-Utsunomiya, K., Mizumura, H., Shefner, J. M., Cox, G. A., Iwakura, Y., Brown, R. H., Jr., and Ikeda, J. -E. (2006) Mice deficient in the Rab5 guanine nucleotide exchange factor ALS2/alsin exhibit age-dependent neurological deficits and altered endosome trafficking. Hum. Mol. Genet. 15 (2), 233-250.
  2. 秦野伸二、池田穰衛(2006)神経系疾患の遺伝子学. 最新医学 61 (9)(増刊号:臨床遺伝子学’06)、1956-1970.


  1. Okada, Y., Sakai, H., Kohiki, E., Suga, E., Yanagisawa, Y., Tanaka, K., Hadano, S., Osuga, H., and Ikeda, J. -E. (2005) A Dopamine D4 receptor antagonist attenuates ischemia-induced neuronal cell damage via upregulation of neuronal apoptosis inhibitory protein. J. Cereb. Blood Flow Metab. 25 (7), 794-806.
  2. Hadano, S. and Ikeda, J. -E. (2005) Purification and functional analyses of ALS2 and its homologue. Methods Enzymol. vol. 403, GTPases Regulating Membrane Targeting and Fusion (Edited by William E. Balch, Channing J. Der, and Alan Hall), Elsevier Inc., San Diego/California, U.S.A., pp310-321.
  3. 秦野伸二(2005) 110. 筋萎縮性側索硬化症原因遺伝子. 予防医学事典、松島綱治、酒井敏行、石川昌、稲寺秀邦 編、朝倉書店、東京、pp251-253.


  1. Hadano, S., Otomo, A., Suzuki-Utsunomiya, K., Kunita, R., Yanagisawa, Y., Showguchi-Miyata, J., Mizumura, H., and Ikeda, J. -E. (2004) ALS2CL, the novel protein highly homologous to the carboxy-terminal half of ALS2, binds to Rab5 and modulates endosome dynamics. FEBS Lett. 575 (1-3), 64-70.
  2. Kunita, R., Otomo, A., Mizumura, H., Suzuki, K., Showguchi-Miyata, J., Yanagisawa, Y., Hadano, S., and Ikeda, J. -E. (2004) Homo-oligomerization of ALS2 through its unique carboxy-terminal regions is essential for the ALS2-associated Rab5 guanine nucleotide exchange activity and its regulatory function on endosome trafficking. J. Biol. Chem. 279 (37), 38626-38635.


  1. Nagano, I., Murakami, T., Shiote, M., Manabe, Y., Hadano, S., Yanagisawa, Y., Ikeda, J. -E., and Abe, K. (2003) Single-nucleotide polymorphisms in uncoding regions of ALS2 gene of Japanese patients with autosomal-recessive amyotrophic lateral sclerosis. Neurol. Res. 25 (5), 505-509.
  2. Otomo, A., Hadano, S., Okada, T., Mizumura, H., Kunita, R., Nishijima, H., Showguchi-Miyata, J., Yanagisawa, Y., Kohiki, E., Suga, E., Yasuda, M., Osuga, H., Nishimoto, T., Narumiya, S., and Ikeda, J. -E. (2003) ALS2, a novel guanine nucleotide exchange factor for the small GTPase Rab5, is implicated in endosomal dynamics. Hum. Mol. Genet. 12 (14), 1671-1687.
  3. 秦野伸二(2003)新しい家族性筋萎縮性側索硬化症遺伝子:ALS2 -運動ニューロン疾患における分子接点.医学のあゆみ 205 (2)、119-123.


  1. Singaraja, R. R., Hadano, S., Metzler, M., Givan, S., Wellington, C. L., Warby, S., Yanai, A., Gutekunst, C. -A., Leavitt, B. R., Yi, H., Fichter, K., Gan, L., McCutcheon, K., Chopra, V., Michel, J., Hersch, S. M., Ikeda, J. -E., and Hayden, M. R. (2002) HIP14, a novel ankyrin domain-containing protein, links huntingtin to intracellular trafficking and endocytosis. Hum. Mol. Genet. 11 (23), 2815-2828.
  2. Okada, T., Gondo, Y., Goto, J., Kanazawa, I., Hadano, S., and Ikeda, J. -E. (2002) Unstable transmission of the RS447 human megasatellite tandem repetitive sequence that contains the USP17 gene encoding a functional deubiquitinating enzyme. Hum. Genet. 110 (4), 302-313.
  3. Xu, M., Okada, T., Sakai, H., Miyamoto, N., Yanagisawa, Y., MacKenzie, A. E., Hadano, S., and Ikeda, J. -E. (2002) Functional human NAIP promoter and transcriptional elements of the human NAIP and psiNAIP genes. Biochim. Biophys. Acta 1574 (1), 35-50.
  4. Kunita, R., Otomo, A., and Ikeda, J. -E. (2002) Identification and characterization of novel members of the CREG family, putative secreted glycoproteins expressed specifically in brain. Genomics 80 (5), 456-460.
  5. 秦野伸二(2002)家族性ALSの新たな遺伝子. BRAIN MEDICAL 14 (1)、47-54.
  6. 秦野伸二(2002)ALSの原因遺伝子. 生化学 74 (6)、483-489.
  7. 秦野伸二(2002)筋萎縮性側索硬化症原因遺伝子の同定とゲノム研究. ゲノム医学 2 (3)、231-240.
  8. 大須賀等、大友麻子、秦野伸二、池田穰衛(2002)ALS2とその原因遺伝子. 最新医学 57 (9)(増刊号:臨床遺伝子学2002)、2237-2249.
  9. 秦野伸二、池田穰衛(2002)家族性筋萎縮性側索硬化症2型(ALS2)の原因遺伝子. 脳機能の解明-生命科学の主潮流-、第8章神経変性疾患の遺伝子異常と病態解明、赤池紀扶、東英穂、阿部康二、久保千春編、ガイア出版会、福岡、pp299-305.


  1. Hadano, S., Hand, C. K., Osuga, H., Yanagisawa, Y., Otomo, A., Devon, R. S., Miyamoto, N., Showguchi-Miyata, J., Okada, Y., Singaraja, R., Figlewicz, D. A., Kwiatkowski, T., Hosler, B. A., Sagie, T., Skaug, J., Nasir, J., Brown, R. H., Jr., Scherer, S. W., Rouleau, G. A., Hayden, M. R., and Ikeda, J. -E. (2001) A gene encoding a putative GTPase regulator is mutated in familial amyotrophic lateral sclerosis 2. Nat. Genet. 29 (2), 166-173.
  2. Hadano, S., Yanagisawa, Y., Skaug, J., Fichter, K., Nasir, J., Martindale, D., Koop, B. F., Scherer, S. W., Nicholson, D. W., Rouleau, G. A., Ikeda, J. -E., and Hayden, M. R. (2001) Cloning and characterization of three novel genes, ALS2CR1, ALS2CR2, and ALS2CR3, in the juvenile amyotrophic lateral sclerosis (ALS2) critical region at chromosome 2q33-q34: Candidate genes for ALS2. Genomics 71 (2), 200-213.
  3. 秦野伸二、池田穰衛(2001)Huntington舞踏病の分子病態. 神経内科 54 (2)、114-121.
  4. 秦野伸二(2001)筋萎縮性側索硬化症(ALS)分子病態研究の最前線. 実験医学 19 (17)、2283-2288.
  5. 秦野伸二(2001)神経変性疾患研究の新たな方向性:GEF. 実験医学 19 (17)、2288.


  1. Matsuyama, N., Hadano, S., Onoe, K., Osuga, H., Showguchi-Miyata, J., Gondo, Y., and Ikeda, J. -E. (2000) Identification and characterization of the miniature pig Huntington's disease gene homolog: evidence for conservation and polymorphism in the CAG triplet repeat. Genomics 69 (1), 72-85.
  2. Saitoh, Y., Miyamoto, N., Okada, T., Gondo, Y., Showguchi-Miyata, J., Hadano, S., and Ikeda, J. -E. (2000) The RS447 human megasatellite tandem repetitive sequence encodes a novel deubiquitinating enzyme with a functional promotor. Genomics 67 (3), 291-300.


  1. Yamamoto, K., Sakai, H., Hadano, S., Gondo, Y., and Ikeda, J. -E. (1999) Identification of two distinct transcripts for the neuronal apoptosis inhibitory protein (NAIP) gene. Biochem. Biophys. Res. Commn. 264 (3), 998-1006.
  2. Hadano, S., Nasir, J., Nichol, K., Rasper, D. M., Vaillancourt, J. P., Sherer, S. W., Beatty, B. G., Ikeda, J. -E., Nicholson, D. W., and Hayden, M. R. (1999) Genomic organization of the human caspase-9 gene on chromosome 1p36.1-p36.3. Mammal. Genome 10 (7), 757-760.
  3. Hadano, S., Nichol, K., Brinkman, R. R., Nasir, J., Martindale, D., Koop, B. F., Nicholson, D. W., Scherer, S. W., Ikeda, J. -E., and Hayden, M. R. (1999) A yeast artificial chromosome-based physical map of the juvenile amyotrophic lateral sclerosis (ALS2) critical region on human chromosome 2q33-q34. Genomics 55 (1), 106-112.


  1. Hadano, S., Ishida, Y., and Ikeda, J. -E. (1998) The primary structure and genomic organization of five novel transcripts located close to the Huntington's disease gene on human chromosome 4p16.3. DNA Res. 5 (3), 177-186.
  2. Rasper, D. M., Vaillancourt, J. P., Hadano, S., Houtzager, V. M., Seiden, I., Keen, S. L. C., Tawa, P., Xanthoudakis, S., Nasir, J., Martindale, D., Koop, B. F., Peterson, E. P., Thornberry, N. A., Huang, J., MacPherson, D. P., Black, S. C., Hornung, F., Lenardo, M. J., Hayden, M. R., Roy, S., and Nicholson, D. W. (1998) Cell death attenuation by 'usurpin', a mammalian DED-caspase homologue that precludes caspase-8 recruitment and activation by the CD-95 (Fas, APO-1) receptor complex. Cell Death Differ. 5 (4), 271-288.


  1. Kogi, M., Fukushige, S., Lefevre, C., Hadano, S., and Ikeda, J. -E. (1997) A novel tandem repeat sequence located on human chromosome 4p: Isolation and characterization. Genomics 42 (2), 278-283.
  2. 秦野伸二、池田穣衛(1997)ダイナミック変異による神経・筋変性疾患. 最新医学 52 (10)、2170-2176.
  3. 秦野伸二、池田穣衛(1997)ハンチントン病遺伝子領域の遺伝子分布と構造. 神経研究の進歩 41 (3)、418-429.


  1. Hadano, S., Ishida, Y., Tomiyasu, H., Yamamoto, K., Bates, G. P., and Ikeda, J. -E. (1996) Transcript map of the human chromosome 4p16.3 consisting of 627 cDNA clones derived from 1 Mb of the Huntington's disease locus. DNA Res. 3 (4), 239-255.
  2. 秦野伸二、池田穣衛(1996)レーザー染色体切断法. 組織培養 22 (2)、55-59.


  1. 秦野伸二(1995)ポジショナルクローニング. 炎症と免疫 3 (3)、324-325.
  2. 秦野伸二、池田穣衛(1995)遺伝子治療の現況と展望. 臨床成人病 25 (1)、26-31.


  1. Ogasawara, M., Seino, T., Hadano, S., Kasugai, A., Ito, A., and Itakura, M. (1994) Exercise-induced prolonged hyperuricemia by continuous production and release of hypoxanthine in fast-twitch muscle in rats. Purine and Pyrimidine Metabolism 18 (1), 1-9.
  2. Ishida, Y., Hadano, S., Nagayama, T., Tomiyasu, H., Wakasa, K., and Ikeda, J. -E. (1994) Isolation and characterization of 21 novel expressed DNA sequences from the distal region of the human chromosome 4p. Genomics 22 (2), 302-312.
  3. Yokoi, H., Hadano, S., Kogi, M., Kang, X., Wakasa, K., and Ikeda, J. -E. (1994) Isolation of expressed sequences encoded by the human Xq terminal portion using microclone probes generated by laser microdissection. Genomics 20 (3), 404-411.


  1. Hadano, S., Ishida, Y., Bates, G. P., Nagayama, T., Kanazawa, I., Lehrach, H., and Ikeda, J. -E. (1993) Generation of high-density DNA markers from yeast artificial chromosome DNA by single unique primer- polymerase chain reaction. Genet. Anal. Techn. Appl. 10 (5), 105-108.
  2. 秦野伸二、池田穣衛(1993)レーザービームを用いた染色体マイクロダイセクション法と疾患遺伝子座領域のクローニング技術. 日本臨牀 51 (9)、24-29 (2240-2245).


  1. Hadano, S., Watanabe, M., Yokoi, H., Kogi, M., Kondo, I., Tsuchiya, H., Kanazawa, I., Wakasa, K., and Ikeda, J. -E. (1991) Laser microdissection and single unique primer PCR allow generation of regional chromosome DNA clones from a single human chromosome. Genomics 11 (2), 364-373.