英　文　原　著　（テーマ別）

ニーマンピック病C型

Kusunoki-Ii M., Kohama H., Kato K., Nomura Y., Nagashima K., Ninomiya H., Kato M. and Kato S. Ultrastructure of spinal anterior horn cells in human Niemann-Pick type C (NPC) patient and mouse model of NPC with retroposon insertion in NPC1 genes.
Pathol. Int. 70, 422-432, 2020

Nakasone N., Nakamura Y.S., Higaki K., Oumi N., Ohno K. and Ninomiya H.
Endoplasmic reticulum-associated degradation of Niemann-Pick C1: evidence for the role of heat shock proteins and identification of lysine residues that accept ubiquitin.
J. Biol. Chem. 289, 19714-19725, 2014

Tani H., Iha K., Shimojima M., Fukushi S., Taniguchi S., Yoshikawa T., Kawaoka Y., Nakasone N., Ninomiya H., Saijo M. and Morikawa S.
Analysis of Lujo virus cell entry using pseudotype vesicular stomatitis virus.
J. Viol. 88, 7317-7330, 2014

Xiong H., Higaki K., Wei C., Bao, X., Zhang Y., Fu N., Qin, J., Adachi K., Kumura Y., Ninomiya H., Nanba E. and Wu X.
Genotype/phenotype of 6 Chinese cases with Niemann-Pick disease type C.
Gene 498, 332-335, 2012

Yanagimoto C., Harada M., Kumemura H., Abe M., Koga H., Sakata M., Kawaguchi T., Terada K., Hanada S., Taniguchi E., Ninomiya H., Ueno T., Sugiyama T. and Sata M.
Copper incorporation into ceruloplasmin is regulated by Niemann-Pick C1 protein.
Hepatol Res. 41, 484-491, 2011

Yanagimoto C., Harada M., Kumemura H., Koga H., Kawaguchi T., Terada K., Hanada S., Taniguchi E., Koizumi Y., Koyota S., Ninomiya H., Ueno T., Sugiyama T. and Sata M.
Niemann-Pick C1 protein transports copper to the secretory compartment from late endosomes where ATP7B resides.
Exp. Cell Res. 315, 119-126, 2009

Luan Z., Saito Y., Miyata H., Ohama E., Ninomiya H. and Ohno K.
Brainstem neuropathology in a mouse model of Niemann-Pick disease type C.
J. Neurol. Sci. 268, 108-116, 2008

Suzuki M., Sugimoto Y., Ohsaki Y., Ueno M., Kato S., Kitamura Y., Hosokawa H., Davies J.P., Ioannou Y.A., Vanier M.T., Ohno K. and Ninomiya H.
Endosomal accumulation of Toll-like receptor 4 causes constitutive secretion of cytokines and activation of STATs in Niemann-Pick disease type C fibroblasts: a potential basis for glial cell activation in the NPC brain.
J. Neurosci. 27, 1879-1891, 2007

Ohsaki Y., Sugimoto Y., Suzuki M., Hosokawa H., Yoshimori T., Davies J.P., Ioannou Y.A., Vanier M.T., Ohno K. and Ninomiya H.
Cholesterol depletion facilitates ubiquitination of NPC1 and its association with SKD1/Vps4.
J. Cell Sci. 199, 2643-2653, 2006

Tamura H., Takahashi T., Ban N., Torisu H., Ninomiya H., Takada G and Inagaki N.
Niemann-Pick type C disease: novel NPC1 mutations and characterization of the concomitant acid sphingomyelinase deficiency.
Mol. Genet. Metab. 87, 113-121, 2006

Harada M., Kawaguchi T., Kumemura H., Terada K., Ninomiya H., Taniguchi E., Hanada S., Maeyama M., Koga H., Ueno T., Furuta K., Suganuma T., Sugiyama T. and Sata M.
The Wilsondisease protein ATP7B resides in the late endosomes with Rab7 and the Niemann-Pick C1 protein.
Am. J. Pathol.166, 499-510, 2005

Ohara S., Ukita Y., Ninomiya H. and Ohno K.
Degeneration of cholecystokinin-immunoreactive afferents to the VPL thalamus of the mouse model of Niemann-Pick type C.
Brain Res.1022, 244-246, 2004

Ohara S., Ukita Y., Ninomiya H. and Ohno K.Axonal dystrophy of dorsal root ganglion sensory neurons in a mouse model of Niemann-Pick disease type C.
Exp. Neurol.187, 289-298, 2004

Ohsaki Y., Sugimoto Y., Suzuki M., Kaidoh T., Shimada Y., Ohno-Iwashita Y., Davies J.P., Ioannou Y.A., Ohno K. and Ninomiya H.
Reduced sensitivity of Niemann-Pick C1-deficient cells to theta-toxin (perfringolysin O): sequestration of toxin to raft-enriched membrane vesicles.
Histochem. Cell Biol. 121, 263-272, 2004

Yamamoto T., Feng J.H., Higaki K., Taniguchi M., Nanba E., Ninomiya H. and Ohno K.
Increased NPC1 mRNA in skin fibroblasts from Niemann-Pick disease type C patients.
Brain Dev. 26, 245-250, 2004

Sugii S., Reid P.C., Ohgami N., Shimada Y., Maue R.A., Ninomiya H., Ohno-Iwashita Y. and Chang T.Y.
Biotinylated theta-toxin derivative as a probe to examine intracellular cholesterol-rich domains in normal and Niemann-Pick type C1 cells.
J. Lipid Res. 44, 1033-1041, 2003

Sugimoto Y., Ninomiya H., Ohsaki Y., Higaki K., Davies J.P., Ioannou Y.A. and Ohno K.
Accumulation of cholera toxin and GM1 ganglioside in the early endosome of Niemann-Pick C1-deficient cells.
Proc. Natl. Acad. Sci. USA 98, 12391-12396, 2001

Taniguchi M., Shinoda Y., Ninomiya H.,Vanier M.T. and Ohno K.
Sites and temporal changes of gangliosides GM1/GM2 storage in the Niemann-Pick disease type C mouse brain.
Brain Dev. 23, 414-421, 2001

Yamada A., Saji M., Ukita Y., Shinoda Y., Taniguchi M., Higaki K., Ninomiya H. and Ohno K.
Progressive neuronal loss in the ventral posterior lateral and medial nuclei of thalamus in Niemann-Pick disease type C mice brain.
Brain Dev. 23, 288-297, 2001

Higaki K., Ninomiya H., Sugimoto Y., Suzuki T., Taniguchi M., Niwa H., Pentchev P.G., Vanier M.T. and Ohno K.
Isolation of NPC1-deficient Chinese hamster ovary cell mutants by gene trap mutagenesis.
J. Biochem. 129, 875-880, 2001

Sawamura N., Gong J.S., Garver W.S., Heidenreich R.A., Ninomiya H., Ohno K., Yanagisawa K. and Michikawa M.
Site-specific phosphorylation of tau accompanied by activation of mitogen-activated protein kinase (MAPK) in brains of Niemann-Pick type C mice.
J. Biol. Chem. 276, 10314-10319, 2001

Tsukamoto H., Yamamoto T., Nishigaki T., Sakai N., Nanba E., Ninomiya H., Ohno K., Inui K. and Okada S.
SSCP analysis by RT-PCR for the prenatal diagnosis of Niemann-Pick disease type C.
Prenat. Diagn. 21, 55-57, 2001

Yamamoto T., Ninomiya H., Matsumoto M., Ohta Y., Nanba E., Tsutsumi Y., Yamakawa K., Millat G., Vanier M.T., Pentchev P.G. and Ohno K.
Genotype-phenotype relationship of Niemann-Pick disease type C: a possible correlation between clinical onset and levels of NPC1 protein in isolated skin fibroblasts.
J. Med. Genet. 37, 707-711, 2000

Yamamoto T., Nanba E., Ninomiya H., Higaki K., Taniguchi M., Zhang H., Akaboshi S., Watanabe Y., Takeshima T., Inui K., Okada S., Tanaka A., Sakuragawa N., Millat G., Vanier M.T., Morris J.A., Pentchev P.G. and Ohno K.
NPC1 gene mutations in Japanese patients with Niemann-Pick disease type C.
J. Hum. Genet. 105, 10-16, 1999


ドウシャン型筋ジストロフィー

Kato Y., Akai A., Suzuki R., Hosokawa H., Ninomiya H., Masaki T., Nagata K. and Tanokura M.
¹H, ¹³C and ¹⁵N assignments of the tandem WW domains of human MAGI-1/BAP-1.
J. Biomol. NMR 29, 539-540, 2004

Hosokawa H., Ninomiya H., Kitamura Y., Fujiwara K. and Masaki T.
Vascular endothelial cells that express dystroglycan are involved in angiogenesis.
J. Cell Sci. 115, 1487-1496, 2002

Shimizu H., Hosokawa H., Ninomiya H., Miner J.H. and Masaki T.
Adhesion of cultured bovine aortic endothelial cells to laminin-1 mediated by dystroglycan.
J. Biol. Chem. 274, 11995-12000, 1999


その他の遺伝性疾患

Yu Y, Mena-Barragan T, Higaki K, Johnson JL, Drury JE, Lieberman RL, Nakasone N, Ninomiya H, Tsukimura T, Sakuraba H, Suzuki Y, Nanba E, Mellet CO, Garcia Fernandez JM, Ohno K.
Molecular basis of 1-deoxygalactonojirimycin arylthiourea binding to human α-galactosidase A: pharmacological chaperoning efficacy on fabry disease mutants.
ACS Chem. Biol. 9, 1460-1469, 2014

Takai T., Higaki K., Moncayo M.A., Barragan T.M., Hirano Y., Yura K., Yu L., Ninomiya H., Moreno M.I.G., Sakakibara Y., Ohno K., Nanba E., Mellet C.O., Fernandez J.M.G. and Suzuki Y.
A bicyclic 1-deoxygalactonojirimycin derivative as a novel pharmacological chaperone for GM1 gangliosidosis.
Mol. Ther. 21, 526-532, 2013

Aguilar-Moncayo M., Takai T., Higaki K., Mena-Barragan T., Hirano Y., Yura K., Li L., Yu Y., Ninomiya H., Garcia-Moreno M.I., Ishii S., Sakakibara Y., Ohno K., Nanba E., Ortiz Mellet C., Garcia Fernandez J.M. and Suzuki Y.
Tuning glycosidase inhibition through aglycone interactions: pharmacological chaperones for Fabry disease and GM(1) gangliosidosis.
Chem. Commun. (Camb) 4, 6514-6516, 2012

Takamura A., Higaki K., Ninomiya H., Takai T., Matsuda J., Iida M., Ohno K., Suzuki Y. and Nanba E.
Lysosomal accumulation of Trk protein in brain of GM1-gangliosidosis mouse and its restoration by chemical chaperone.
J. Neurochem.118, 399-406, 2011

Higaki K., Li L., Bahrudin U., Okuzawa S., Takamura A., Yamamoto K., Adachi K., Paraguison R. C., Takai T., Ikehata H., Tominaga L., Hisatome I., Iida M., Ogawa S., Matsuda J., Ninomiya H., Sakakibara Y., Ohno K., Suzuki Y. and Nanba E.
Chemical chaperone therapy: chaperone effect on mutant enzyme and cellular pathophysiology in beta-galactosidase deficiency.
Human Mut. 32, :843-852, 2011

Luan Z., Higaki K., Aguilar-Moncayo M., Li L., Ninomiya H., Nanba E., Ohno K., Garcia-Moreno M.I., Ortiz M.C., Garcia Fernandez J.M. and Suzuki Y.
A fluorescent sp²-iminosugar with pharmacological chaperone activity for gaucher disease: synthesis and intracellular distribution studies.
Chembiochem.11, 2453-2464, 2010

Li L., Higaki K., Ninomiya H., Luan Z., Iida M., Ogawa S., Suzuki Y., Ohno K. and Nanba E.
Chemical chaperone therapy: Luciferase assay for screening of β-galactosidase mutations.
Mol. Genet. Metab. 101, 364-369, 2010

Luan Z., Ninomiya H., Ohno K., Ogawa S., Kubo T., Iida M. and Suzuki Y.
The effect of N-octyl-β-valienamine on beta-glucosidase activity in tissues of normal mice.
Brain Dev. 32, 805-809, 2010

Luan Z., Li L., Ninomiya H., Ohno K., Ogawa S., Kubo T., Iida M. and Suzuki Y.
The pharmacological chaperone effect of N-octyl-β-valienamine on human mutant acid β-glucosidases.
Blood Cells Mol. Diseases 44, 48-54, 2010

Luan Z., Higaki K., Aguilar-Moncayo M., Ninomiya H., Ohno K., Garcia-Moreno M.I., Ortiz Mellet C., Garcia Fernandez J.M. and Suzuki Y.
Chaperone activity of bicyclic nojirimycin analogues for Gaucher mutations in comparison with N-(n-nonyl)deoxynojirimycin.
Chembiochem. 23, 2780-2792, 2009

Takamura A., Higaki K., Kajimaki K., Otsuka S., Ninomiya H., Matsuda J., Ohno K., Suzuki Y. and Nanba E.
Enhanced autophagy and mitochondrial aberrations in murine GM1-gagliosidosis.
Biochem. Biophys. Res. Commun. 367, 616-622, 2008

Saito Y., Toyoshima M., Oka A., Luan Z., Moriwaki S., Yamamoto O., Kanzaki S., Hanaki K., Ninomiya H., Nanaba E., Kondo A., Maegaki Y. and Ohno K.
Mental retardation, spasticity, basal ganglia calcification, cerebral white matter lesions, multiple endocrine defects, telangiectasia and atrophic skin: A new syndrome?
Brain Dev. 30, 221-225, 2007

Floricel F. Higaki KMaki HNanba E., Ninomiya H. and Ohno K.
Antisense suppression of TSC1 gene product, hamartin, enhances neurite outgrowth in NGF-treated PC12h cells.
Brain Dev. 29, 502-509, 2007

Yasui S., Tsuzaki K., Ninomiya H., Floricel F., Asano Y., Maki H., Takamura A., Nanba E., Higaki K. and Ohno K.
The TSC1 gene product hamartin interacts with NADE.
Mol Cell Neurosci. 35, 100-108, 2007

Lei K., Ninomiya H., Suzuki M., Inoue T., Sawa M., Ida H., Eto Y., Ogawa S., Ohno K.
Enzytme enhancement activity of N-Octyl-beta-valienamine on beta-glucosidase mutants associated with Gaucher disease.
Biochim. Biophys. Acta 1772, 587-596, 2007

Kawashima Y., Kanzaki S., Yang F., Kinoshita T., Hanaki K., Nagaishi J.I., Ohtsuka Y., Hisatome I., Ninomiya H., Nanba E., Fukushima T. and Takahashi S.I..
Mutation at the cleavage site of IGF receptor in short stature children born with intrauterine growth retardation.
J. Clin. Endocrinol. Metab. 90, 4679-4687, 2005

Hou L., Sugimoto Y., Ohsaki Y., Ninomiya H., Oka A., Taniguchi M., Ida H., Eto Y., Ogawa S., Matsuzaki Y., Sawa M., Inoue T., Higaki K., Nanba E., Ohno K. and Suzuki Y.
N-Octyl-beta-valienamine up-regulates activity of F213I mutant beta-glucosidase in cultured cells: a potential chemical chaperone therapy for Gaucher disease.
Biochim. Biophys. Acta 1689, 219-228, 2004

Feng J.H., Yamamoto T., Nanba E., Ninomiya H., OkaA., and Ohno K.
Novel TSC2 mutations and decreased expression of tuberin in cultured tumor cells with an insertion mutation.
Hum. Mutat. 24, 397, 2004

Pipo J.R., Feng J.H., Yamamoto T., Ohsaki Y., Nanba E., Tsujino S., Sakuragawa N., Martiniuk F., Ninomiya H., Oka A. and Ohno K.
New GAA mutations in Japanese patients with GSDII (pompe disease).
Pediatr Neurol. 29, 284-287, 2003

Matsuzawa F., Aikawa S.I., Sakuraba H., Tanaka A., Ohno K., Ninomiya H., Sugimoto Y. and Doi H.
Structural basis of GM2 gangliosidosis B variant.
J. Hum. Genet. 48, 582-589, 2003

Yamamoto T., Pipo J.R., Feng J.H., Takeda H., Nanba E., Ninomiya H., and Ohno K.
Novel TSC1 and TSC2 mutations in Japanese patients with tuberous sclerosis complex.
Brain Dev. 24, 227-230, 2002

Yamamoto T., Pipo J.R., Ninomiya H., Ieshima A. and Koeda T.
Antley-Bixler syndrome and maternal virilization: a proposal of genetic heterogeneity.
Clin. Genet. 59, 451-453, 2001

Pipo J.R., Yamamoto T., Takeda H., Maegawa S., Nanba E., Ninomiya H., Ohno K. and Takeshita K.
Two novel serine repeat length polymorphisms (1043insS and 1043inssS) at exon 23 of the TSC1 gene.
Hum. Mutat. 16, 375, 2000

Chikumi H., Yamamoto T., Ohta Y., Nanba E., Nagata K., Ninomiya H., Narasaki K., Katoh T., Hisatome I., Ono K., Tanaka Y., Kuroda H. and Ohgi S.
Fibrillin gene (FBN1) mutations in Japanese patients with Marfan syndrome.
J. Hum. Genet. 45, 115-118, 2000

Yamamoto T., Akaboshi S., Ninomiya H. and Nanba E.
DEFECT 11 syndrome associated with agenesis of corpus callosum.
J. Med. Genet. 38, E5, 2000

Nagata K., Yamamoto T., Chikumi H., Ikeda T., Yamamoto H., Hashimoto K., Yoneda K., Nanba E., Ninomiya H. and Ishitobi K.
A novel interstitial deletion of KAL1 in a Japanese family with Kallmann syndrome.
J. Hum. Genet. 45, 237-240, 2000

Zhang H., Nanba E., Yamamoto T., Ninomiya H., Ohno K., Mizuguchi M. and Takeshita K.
Mutational analysis of TSC1 and TSC2 genes in Japanese patients with tuberous sclerosis complex.
J. Hum. Genet. 44, 391-393, 1999


アルツハイマー病

Uemura K., Kuzuya A., Aoyagi N., Ando N., Ando K., Shimozono Y., Ninomiya H., Shimohama S. and Kinoshita A.
Amyloid beta inhibits ectodomain shedding of N-cadherin via down-regulation of cell-surface NMDA receptor.
Neuroscience 145, 5-10, 2007

Kuzuya A., Uemura K., Kitagawa N., Aoyagi N., Kihara T., Ninomiya H., Ishiura S., Takahashi R. and Shimohama S.
Presenilin 1 is involved in the maturation of beta-site amyloid precursor protein-cleaving enzyme 1 (BACE1).
J. Neurosci. Res. 85, 153-165, 2007

Uemura K., Kihara T., Kuzuya A., Okawa K., Nishimoto T., Bito H., Ninomiya H., Sugimoto H., Kinoshita A. and Shimohama S.
Activity-dependent regulation of beta-catenin via epsilon-cleavage of N-cadherin.
Biochem. Biophys. Res. Commun. 345, 951-958, 2006

Uemura K., Kihara T., Kuzuya A., Okawa K., Nishimoto T., Ninomiya H., Sugimoto H., Kinoshita A and Shimohama S.
Characterization of sequential N-cadherin cleavage by ADAM10 and PS1.
Neurosci. Lett. 402, 278-283, 2006

Wakutani Y., Watanabe K., Adachi Y., Wada-Isoe K., Urakami K., Ninomiya H., Saido T.C., Hashimoto T., Iwatsubo T. and Nakashima K.
Novel amyloid precursor protein gene missense mutation (D678N) in probable familial Alzheimer’s disease.
J. Neurol. Neurosurg. Psychiatry 75, 1039-1042, 2004

Jin L.W., Ninomiya H., Roch J.M., Schubert D., Masliah E., Otero D.A. and Saitoh T.
Peptides containing the RERMS sequence of amyloid beta/A4 protein precursor bind cell surface and promote neurite extension.
J. Neurosci. 14, 5461-5470, 1994

Ninomiya H., Roch J.M., Jin L.W. and Saitoh T.
Secreted form of amyloid /A4 precursor (APP) binds to two distinct APP binding sites on rat B103 neuron-like cells through two different domains, but only one site is involved in neuritotrophic activity.
J. Neurochem. 63, 495-500, 1994

Ninomiya H, Roch J.M., Sundsmo M.P., Otero D.A.C. and Saitoh T.
Amino acid sequence RERMS represents the active domain of amyloid /A4 protein precursor that promotes fibroblast growth.
J. Cell Biol. 121, 879-886, 1993

Ninomiya H, Fukunaga R., Taniguchi T., Fujiwara M., Shimohama S. and Kameyama M.
[³H]N-[1-(2-Thienyl)cyclohexyl]-3,4-piperidine ([³H]TCP) binding in human frontal cortex: decreases in Alzheimer-type dementia.
J. Neurochem. 54, 526-532, 1990


循環器系の共同研究@鳥取

Ueno S., Hamada T., Taniguchi S., Ohtani N., Miyazaki S., Mizuta E., Ohtahara A., Ogino K., Yoshida A., Kuwabara M., Yoshida K., Ninomiya H., Kotake H., Taufiq F., Yamamoto K. & Hisatome I.
Effect of Antihypertensive Drugs on Uric Acid Metabolism in Patients with Hypertension: Cross-Sectional Cohort Study.
Drug. Res. in press

Mahati E., Li P., Kurata Y., Maharani N, Nobuhito I., Sakata S., Ogura K., Miake J., Aiba T., Shimizu W., Nakasone N., Ninomiya H., Higaki K., Yamamoto K., Nakai A., Shirayoshi Y. & Hisatome I.
M3 muscarinic receptor signaling stabilizes a novel mutant hERG channel protein via phosphorylation of HSF1 in transfected cells.
Cir. J. in press

Sogo T., Morikawa K., Kurata Y., Li P., Ichinose T., Yuasa S., Nozaki D., Miake J., Ninomiya H., Shimizu W., Fukuda K., Yamamoto K., Shirayoshi Y. and Hisatome I.
Electrophysiological properties of iPS cell-derived cardiomyocytes from a patient with long QT syndrome type 1 harboring the novel mutation M437V of KCNQ1.
Regenerative Ther. in press

Mishima M., Hamada T., Maharani N., Ikeda N., Onohara T., Notsu T., Ninomiya H., Miyazaki S., Mizuta E., Sugihara S., Kato M., Ogino K., Kuwabara M., Hirota Y., Yoshida A., Otani N., Anzai N. and Hisatome I.
Effects of Uric Acid on the NO Production of HUVECs and its Restoration by Urate Lowering Agents.
Drug Res. 66, 270-274, 2016

Kondo T., Hisatome I., Yoshimura S., Mahati E., Notsu T., Li P., Iitsuka K., Kato K., Ogura K., Miake J., Aiba T., Shimizu W., Kurata Y., Sakata S., Nakasone N., Ninomiya H., Nakai A., Higaki K. Kawata Y., Shirayoshi Y., Yoshida A. and Yamamoto K.
Characterization of the novel mutant A78T-HERG from a long QT syndrome type 2 patient: instability of the mutant protein and stabilization by heat shock factor 1.
J. of Arrhythmia 32, 433-440, 2016

Endo R., Bahrudin U., Notsu T., Tanno S., Onohara T., Yamaguchi S., Ikeda N., Surastri B., Nakayama Y., Ninomiya H., Shirayoshi Y., Inagaki Y., Yamamoto K., Yoshida A. and Hisatome I.
Carvedilol suppresses apoptosis and ion channel remodelling of HL-1 cardiac myocytes expressing E334K cMyBPC.
Drug Res. 66, 126-129, 2016

Maharani N, Ting Y., Cheng J., Hasegawa A., Kurata Y., Li P., Nakayama Y., Ninomiya H., Ikeda N., Morikawa K., Yamamoto K., Makita N., Yamashita T., Shirayoshi Y. and Hisatome I.
Molecular mechanisms underlying urate-induced enhancement of Kv1.5 channel expression in HL-1 atrial myocytes.
Cir. J.79, 2659-2668, 2015

Endo R., Kurata Y., Notsu T., Li P., Morikawa K., Kondo T., Ogura K., Miake J., Yoshida A., Shirayoshi Y., Ninomiya H., Higaki K., Kuwabara M., Yamamoto K., Inagaki Y. and Hisatome I.
Stabilization of Kv1.5 channel protein by the inotropic agent olprinone.
Eur. J. Pharmacol. 765, 488-494, 2015

Li P., Kurata Y., Maharani N., Mahati E., Higaki K., Hasegawa A., Shirayoshi Y., Yoshida A., Kondo T., Kurozawa Y., Yamamoto K., Ninomiya H. and Hisatome I.
E3 ligase CHIP and Hsc70 regulate Kv1.5 protein expression and function in mammalian cells.
J. Mol. Cell. Cardiol. 86, 138-146, 2015

Sugihara S., Hisatome I., Kuwabara M., Niwa K., Maharani N., Kato M., Ogino K., Hamada T., Ninomiya H., Higashi Y., Ichida K. and Yamamoto K.
Depletion of uric acid due to SLC22A12 (URAT1) loss-of-function mutation causes endothelial dysfunction in hypouricemia.
Circ. J. 79, 1125-1132, 2015

Utami S.B., Mahati E., Li P., Maharani N., Ikeda N., Bahrudin U., Munemura C., Hosoyamada M., Yamamoto Y., Yoshida A., Nakayama Y., Higaki K., Nanba E., Ninomiya H., Shirayoshi Y., Ichida K., Yamamoto K., Hosoya T. and Hisatome I.
Apoptosis induced by an uromodulin mutant C112Y and its suppression by topiroxostat.
Clin. Exp. Nephrol. in press

Otsuki Y., Nakamura Y., Harada S., Yamamoto Y., Ogino K., Morikawa K., Ninomiya H., Miyagawa S., Sawa Y., Hisatome I. and Nishimura M.
Adipose stem cell sheets improved cardiac function in the rat myocardial infarction, but did not alter cardiac contractile responses to β-adrenergic stimulation.
Biomed. Res. 36, 11-19, 2015

Sakata S, Kurata Y, Li P, Notsu T, Morikawa K, Miake J, Higaki K, Yamamoto Y, Yoshida A, Shirayoshi Y, Yamamoto K, Horie M, Ninomiya H, Kanzaki S and Hisatome I.
Instability of KCNE1-D85N that causes long QT syndrome: stabilization by verapamil.
Pacing. Clin. Electrophysiol. 37, 853-863, 2014

Hamada T., Kuwabara M., Watanabe A., Mizuta E., Ohtahara A., Omodani H., Watanabe M., Nakamura H., Hirota Y., Miyazaki S., Kato M., Ogino K., Kosaka H., Ninomiya H., Taniguchi S., Yamamoto K., Kotake H. and Hisatome I. (2014) A comparative study on the effectiveness of losartan/hydrochlorothiazide and telmisartan/hydrochlorothiazide in patients with hypertension. Clin. Exp. Hypertens. 36, 251-257, 2014

Miyazaki S, Hamada T, Hirata S, Ohtahara A, Mizuta E, Yamamoto Y, Kuwabara M, Nosaka Y, Igawa O, Ogino K, Kato M, Yoshida A, Ninomiya H, Cheng J, Moriwaki Y, Yamamoto K, and Hisatome I.
Effects of azelnidipine on uric acid metabolism in patients with essential hypertension.
Clin. Exp. Hypertens. 36, 447-453

Nindita Y, Hamada T, Bahrudin U, Hosoyamada M, Ichida K, Iwai C, Urashima S, Kuwabara M, Utami SB, Mizuta E, Yamada K, Igawa O, Shigemasa C, Ninomiya H, Tsuchihashi T, Hisatome I.
Effect of losartan and benzbromarone on the level of human urate transporter 1 mRNA.
Drug Res. (Stuttg) 64, 103, 2014

Iwai C., Li P., Kurata Y., Hoshikawa Y., Morikawa K., Maharani N., Higaki K., Sasano T., Notsu T., Ishido Y., Miake J., Yamamoto Y., Shirayoshi Y., Ninomiya H., Nakai A., Murata S., Yoshida A., Yamamoto K., Hiraoka M. and Hisatome I.
Hsp90 prevents interaction between CHIP and HERG proteins to facilitate maturation of wild-type and mutant HERG proteins.
Cardiovasc. Res.100, 520-528, 2013

Bahrudin U., Ikeda N., Utami S.B., Maharani N., Morikawa K., Li P., Sobirin M.A., Hasegawa A., Sakata S., Endo R., Rifqi S., Shirayoshi Y., Yamamoto K., Ninomiya H. and Hisatome I. (2013) Simultaneous treatment with azelnidipine and olmesartan inhibits apoptosis of Hl-1 cardiac myocytes expressing E334K cMyBPC. Drug Res. (Stuttg) 63, 515-520, 2013

Li P., Ogino K., Hoshikawa Y., Morisaki H., Toyama K., Morisaki T., Morikawa K., Ninomiya H., Yoshida A., Hashimoto K., Shirayoshi Y. and Hisatome I.
AMP deaminase 3 plays a critical role in remote reperfusion lung injury.
Biochem. Biophys. Res. Commun. 434, 131-136, 2013

Suzuki S., Kurata Y., Li P., Notsu T., Hasegawa A., Ikeda N., Kato M., Miake J., Sakata S., Shiota G., Yoshida A., Ninomiya H., Higaki K., Yamamoto K., Shirayoshi Y. and Hisatome I.
Stabilization of Kv1.5 channel protein by bepridil through its action as a chemical chaperone.
Eur. J. Pharmacol. 696, 28-34, 2012　

Fujii H., Ikeuchi Y., Kurata Y., Ikeda N., Bahrudin U., Li P., Nakayama Y., Endo R., Hasegawa A., Morikawa K., Miake J., Yoshida A., Hidaka K., Morisaki T., Ninomiya H., Shirayoshi Y., Yamamoto K. and Hisatome I.
Electrophysiological properties of prion-positive cardiac progenitors derived from murine embryonic stem cells.
Cir. J. 76, 2875-2883, 2012

Mizuta E., Utami S.B., Ohtahara A., Endo S., Mishima M., Hasegawa A., Yamada K., Kato M., Yamamoto K., Ogino K., Ninomiya H., Miyazaki S., Hamada T., Taniguchi S.I., Cheng J. and Hisatome I.
A vasodilating β1 blocker celiprolol inhibits muscular release of uric acid precursor in patients with essential hypertension.　
Horm Metab Res. 45, 69-73, 2012

Hamada T., Yamada K., Mizuta E., Watanabe A., Osaki T., Ishida K., Hasegawa A., Sakata S., Mishima M., Ogino K., Nosaka Y., Miyazaki S., Ohtahara A., Ninomiya H., Kato M., Yoshida A., Taniguchi S.I., Yamamoto K. and Hisatome I.
Effects of cilnidipine on srum uric acid level and urinary nitrogen monoxide excretion in patients with hypertension..
Clin. Exp. Hypertens. 34, 470-473, 2012

Bahrudin U., Morikawa K.,Takeuchi A., Kurata Y., Miake J., Mizuta E., Adachi K., Higaki K., Yamamoto Y., Shirayoshi Y., Yoshida A., Kato M., Yamamoto K., Nanba E., Morisaki H., Morisaki T., Matsuoka S., Ninomiya H. and Hisatome I.
Impairment of ubiquitin-proteasome system by E334K cMyBPC modifies channel proteins leading to electrophysiological dysfunction.
J. Mol. Biol.413, 857-878, 2011

Li P., Tanaka S., Ichiyanagi T., Ninomiya H., Ting Y., Utami S.B., Aimi T., Shirayoshi Y., Miake J., and Hisatome I.
Novel effects of extracts from poisonous mushrooms on expression and function of the human ether-a-go-go-related gene channel.
Biol. Pharm. Bull. 34, 1474-1480, 2011

Ting Y., Morikawa K., Kurata Y., Li P., Bahrudin U., Mizuta E., Kato M., Miake J., Yamamoto Y., Yoshida A., Murata M., Inoue T., Nakai A., Shiota G., Higaki K., Nanba E., Ninomiya H., Shirayoshi Y. and Hisatome I.
Transcriptional activation of the anchoring protein SAP97 by heat shock factor (HSF)-1 stabilizes K(v) 1.5 channels in HL-1 cells.
Br. J. Pharmacol. 162, 1832-1842, 2011

Li P., Ninomiya H., Kurata Y., Kato M., Miake J., Yamamoto Y., Igawa O., Nakai A., Higaki K., Toyoda F., Wu J., Horie M., Matsuura H., Yoshida A., Shirayoshi Y., Hiraoka M. and Hisatome I..
Reciprocal control of hERG stability by Hsp70 and Hsc70 with implication for restoration of LQT2 mutant stability.
Cir. Res. 108, 458-468, 2011

Mizuta E., Shirai M., Arakawa K., Hidaka K., Miake J., Ninomiya H., Kato M., Shigemasa C., Shirayoshi Y., Hisatome I. and Morisaki T.
Different distribution of Cav3.2 and Cav3.1 transcripts encoding T-type Ca(2+) channels in the embryonic heart of mice.
Biomed. Res. 31, 301-305, 2010

Nindita Y., Hamada T., Bahrudin U., Hosoyamada M., Ichida K., Iwai C., Urashima S., Kuwabara N., Utami S.B., Mizuta E., Yamada K., Igawa O., Shigemasa C., Ninomiya H., Tsuchihashi T. and Hisatome I.
Effect of losartan and benzbromarone on the level of human urate transporter 1 mRNA. Arzneimittelforschung 60, 186-188, 2010

Hamada T., Mizuta E., Kondo T., Hirai M., Yamada K., Kato M., Shigemasa C., Yamamoto Y., Ninomiya H., Igawa O. and Hisatome I.
Effects of a low-dose antihypertensive diuretic in combination with losartan, telmisartan, or candesartan on serum urate levels in hypertensive patients.
Arzneimittelforschung 60, 71-75, 2010

Koshida S., Kurata Y., Notsu T., Hirota Y., Kuang T-Y., Li P., Bahrudin U., Harada S., Miake J., Yamamoto Y., Hoshikawa Y., Igawa O, Higaki K., Soma M., Yoshida A., Ninomiya H., Shiota G., Shirayoshi Y. and Hisatome I.
Stabilizing effects of eicosapentaenoic acid on Kv1.5 channel protein expressed in mammalian cells.
Eur. J. Pharmacol. 604, 93-102, 2009

Bahrudin U., Morisaki H., Morisaki T., Ninomiya H., Nanba E., Igawa O., Mizuta E., Miake J., Yamamoto Y., Shirayoshi Y., Kitakaze M., Carrier L., Hisatome I., Higaki K. and Takashima S.
Ubiquitin-proteasome system impairment caused by a missense cardiac myosin-binding protein C mutation and associated with cardiac dysfunction in hypertrophic cardiomyopathy.
J Mol. Biol. 384, 896-907, 2008

Yano S., Miake J., Mizuta E., Manabe K., Bahrudin U., Morikawa K., Arakawa K., Sasaki N., Igawa O., Shigemasa C., Yamamoto Y., Morisaki T., Hidaka K., Kurata Y., Yoshida A., Shiota G., Higaki K., Ninomiya H., Lee J. K., Shirayoshi Y. and Hisatome I.
Changes of HCN gene expression and I(f) currents in Nkx2.5-positive cardiomyocytes derived from murine embryonic stem cells during differentiation.
Biomed. Res. 29, 195-203, 2008

Hamada T., Ichida K., Hosoyamada M., Mizuta E., Yanagihara K., Sonoyama K., Sugihara S., Igawa O., Hosoya T., Ohtahara A., Shigamasa C., Yamamoto Y., Ninomiya H. and Hisatome I.
Uricosuric action of losartan via the inhibition of urate transporter 1 (URAT 1) in hypertensive patients.
Am. J. Hypertens. 21, 1157-1162, 2008

Hirota Y., Kato M., Notsu T., Koshida S., Inoue T., Kawata Y., Miake J., Bahrudin U., Li P., Hoshikawa Y., Yamamoto Y., Igawa O., Shirayoshi Y., Nakai A., Ninomiya H., Higaki K., Hiraoka M. and Hisatome I.
Functional stabilization of Kv1.5 protein by Hsp70 in mammalian cell lines.
Biochem. Biophys. Res. Commun. 372, 469-474, 2008

Ogura K., Miake J., Sasaki N., Iwai C., Bahrudin U., Li P., Kato M., Iitsuka K., Hirota Y., Koshida T., Yamamoto Y., Inoue Y., Yano A., Adachi M., Igawa O., Kurata Y., Morisaki T., Shiota G., Shirayoshi Y., Ninomiya H. and Hisatome I.
Inhibition of beta-adrenergic signaling by intracellular AMP is independent of cell-surface adenosine receptors in rat cardiac cells.
J. Mol. Cell. Cardiol. 43, 648-652, 2007

Li P., Ogino K., Hoshikawa Y., Morisaki H., Cheng J., Toyama K., Morisaki T.,Hashimoto K., Ninomiya H., Tomikura-Shimoyama Y., Igawa O., Shigemasa C. and Hisatome I..
Remote reperfusion lung injury is associated with AMP deaminase 3 activation and attenuated by inosine monophosphate.
Circ J. 71, 591-596, 2007

Mizuta E., Furuichi H., Kazuki Y., Miake J., Yano S., Bahrudin U., Yamamoto Y., Igawa O., Shigemasa, C., Hidaka K., Morisaki T., Kurata Y., Ninomiya H., Kitakaze M., Shirayoshi Y., Oshimura M. and Hisatome I..
Delayed onset of beating and decreased expression of T-type Ca²⁺ channel in mouse ES cell-derived cardiocytes carrying human chromosome 21.
Biochem. Biophys. Res. Commun. 351, 126-132, 2006

Kato M., Ogura K., Miyake J., Sasaki N., Taniguchi S., Igawa O., Yoshida A., Shigemasa C., Murata M., Nanba E., Kurata Y., Morisaki T., Ninomiya H. and Hisatome I..
Evidence for proteasomal degradation of Kv1.5 channel protein.
Biochem. Biophys. Res. Commun. 337, 343-348, 2005

Mizuta E., Miake J., Yano S., Furuichi H., Manabe K., Igawa O., Hoshiakwa Y., Nanba E., Ninomiya H., Morisaki T. and Hisatome I..
Subtype switch of T-type Ca²⁺ channels from Cav3.1 to Cav3.2 during differentiation of embryonic stem cells to cardiac lineage cells.
Circ. J. 69, 1284-1289, 2005

Sonoyama K., Igawa O., Miake J., Yamamoto Y., Sugihara S., Sasaki N., Shimoyama M., Hamada T., Taniguchi S., Yoshida A., Ogino K., Shigemasa C., Kurata Y., Shiota G., Narahashi T., Horiuchi M., Matsubara H., Ninomiya H. and Hisatome I..
Effects of angiotensin II on the action potential durations of atrial myocytes in hypertensive rats.
Hypertens. Res. 28, 173-179, 2005

Tanaka H., Miake J., Notsu T., Sonyama K, Sasaki N., IItsuka K., Kato M., Taniguchi S., Igawa O., Yoshida A., Shigemasa C., Hoshikawa H., Kurata Y., Kuniyasu A., Nakayama H., Inagaki N., Nanba E., Shiota G., Morisaki T., Ninomiya H., Kitakaze M. and Hisatome I..
Proteasomal degradation of Kir6.2 channel protein and its inhibition by a Na⁺ channel blocker aprindine.
Biochem. Biophys. Res. Commun. 331, 1001-1006, 2005


エンドセリン@京都

Okamoto Y., Ninomiya H., Miwa S. and Masaki T.
Cholesterol oxidation switches the internalization pathway of endothelin receptor type A from caveolae to clathrin-coated pits in Chinese hamster ovary cells.
J. Biol. Chem. 275, 6439-6446, 2000

Inui T., Ninomiya H., Sasaki Y., Makatani M., Urade Y., Masaki T. and Yamamura T.
Selective activation of excitation-contraction coupling pathways by ETA and ETB receptors in guinea-pig tracheal smooth muscle.
Br. J. Pharmacol. 126, 893-902, 1999

Okazawa M., Shiraki T., Ninomiya H., Kobayashi S. and Masaki T.
Endothelin-induced apoptosis of A375 melanoma cells.
J. Biol. Chem. 273, 12584-12592, 1998

Ninomiya H., Inui T. and Masaki T.
Paracrine endothelin signaling in the control of basal cell proliferation in guinea pig tracheal epithelium.
J. Pharmacol. Exp. Ther. 286, 469-480, 1998

Zhang X.F., Komuro T., Miwa S., Minowa T., Iwamuro Y., Okamoto Y., Ninomiya H., Sawamura T. and Masaki T.
Role of nonselective cation channels as Ca²⁺ entry pathway in endothelin-1-induced contraction and their suppression by nitric oxide.
Eur. J. Pharmacol. 352, 237-245, 1998

Komuro T., Miwa S., Zhang X.F., Minowa T., Enoki T., Kobayashi S., Okamoto Y., Ninomiya H., Sawamura T., Kikuta K., Iwamuro Y., Furutani H., Hasegawa H., Uemura Y., Kikuchi H. and Masaki T.
Physiological role of Ca²⁺-permiable nonselective cation channel in endothelin-1-induced contraction of rabbit aorta.
J. Cardiovasc. Pharmacol. 30, 504-509, 1997

Komuro T., Miwa S., Minowa T., Okamoto Y., Enoki T., Ninomiya H., Zhang X.F., Uemura Y., Kikuchi H. and Masaki T.
The involvement of a novel mechanism distinct from the thrombin receptor in the vasoconstriction induced by trypsin.
Br. J. Pharmacol. 120, 851-856, 1997

Ohtani T., Ninomiya H., Okazawa M., Imamura S. and Masaki T.
Bromodeoxyuridine-induced expression of endothelin A in A375 human melanoma cells.
Biochem. Biophys. Res. Commun. 234, 526-530, 1997

Okamoto Y., Ninomiya H., Tanioka M., Sakamoto A., Miwa S. and Masaki T.
Palmitoylation of human endothelin B: its critical role in G protein coupling and a differential requirement for the cytoplasmic tail by G protein subtypes.
J. Biol. Chem. 272, 21589-21596, 1997

Sugawara F., Ninomiya H., Okamoto Y., Miwa S., Mazda O., Katsura Y. and Masaki T.
Endothelin-1-induced mitogenic responses of Chinese hamster ovary cells expressing human endothelin A: the role of a wortmannin-sensitive signaling pathway.
Mol. Pharmacol. 49, 447-457, 1996

Takagi Y., Ninomiya H., Sakamoto A., Miwa S. and Masaki T.
Structural basis of G protein specificity of human endothelin receptors: a study with endothelin A/B chimeras.
J. Biol. Chem. 270, 10072-10078, 1995

Fujitani Y., Ninomiya H., Okada T., Urade Y. and Masaki T.
Suppression of endothelin-1-induced mitogenic responses of human aortic smooth muscle cells by interleukin-1beta.
J. Clin. Invest. 95, 2474-2482, 1995

Okamoto Y., Ninomiya H., Miwa S. and Masaki T.
Capacitative Ca²⁺ entry in human platelets is resistant to nitric oxide.
Biochem. Biophys. Res. Commun. 212, 90-96, 1995

Enoki T., Miwa S., Sakamoto A., Minowa T., Komuro T., Kobayashi S., Ninomiya H. and Masaki T.
Long-lasting activation of cation current by low concentration of endothelin-1 in mouse fibroblasts and smooth muscle cells of rabbit aorta.
Br. J. Pharmacol. 115, 479-485, 1995


アミノ酸トランスポーター

Hosokawa H., Ninomiya H., Sawamura T., Sugimoto Y., Ichikawa A., Fujiwara K. and Masaki T.
Neuron-specific expression of cationic amino acid transporter 3 in the adult rat brain.
Brain Res. 838, 158-165, 1999

Hosokawa H., Sawamura T., Kobayashi S., Ninomiya H., Miwa S. and Masaki T.
Cloning and characterization of a brain-specific cationic amino acid transporter.
J. Biol. Chem. 272, 8717-8722, 1997


レセプター・シグナル

Higaki K., Ninomiya H., Saji M., Maki H., Koike T. and Ohno K.
Protective effect of neutropin against LPS-induced hypotension and lethality linked to suppression of iNOS induction.
Japan. J. Pharmacol. 86, 329-335, 2001

Taniguchi T., Ninomiya H., Fukunaga R., Ebii K., Yamamoto M. and Fujiwara M.
Neurokinin A-stimulated phosphoinositide breakdown in rabbit iris sphincter muscle.
Japan. J. Pharmacol. 59, 213-220, 1991

Fukunaga R., Ninomiya H., Shimohama S., Kimura J., Kameyama M. and Taniguchi T.
Reassessment of [³H]glutamate binding to human brain membrane preparations.
Japan. J. Pharmacol. 55, 191-196, 1991

Ninomiya H. and Fujiwara M.
Epithio-11,12-methano-thromboxane A₂ stimulates inositol phosphates accumulation in isolated canine mesenteric artery strips.
Japan. J. Pharmacol. 57, 349-354, 1991

Ninomiya H., Taniguchi T. and Fujiwara M.
Phosphoinositide breakdown in rat hippocampal slices: sensitivity to glutamate induced by in vitro anoxia.
J. Neurochem. 55, 1001-1007, 1990

Ninomiya H, Taniguchi T., Fujiwara M. and Kameyama M.
Effects of oxygen depletion on norepinephrine- and carbachol-stimulated phosphoinositide turnover in rat brain slices.
J. Neurochem. 53, 183-190, 1989

Ninomiya H., Taniguchi T., Fujiwara M., Shimohama S. and Kameyama M.
Effects of in vivo exposure to hypoxia on muscarinic cholinergic receptor-coupled phosphoinositide turnover in the rat brain.
Brain Res. 482, 109-121, 1989

Nishikawa H., Taniguchi T., Ninomiya H. and Fujiwara M.
Nicotinic acetylcholine receptors in the rat stomach:II Alterations after vagotomy.
Eur. J. Pharmacol. 146, 105-111, 1988

Nishikawa H., Taniguchi T., Ninomiya H. and Fujiwara M.
Nicotinic acetylcholine receptors in the rat stomach:I (-)-[³H]Nicotine binding.
Eur. J. Pharmacol. 146, 97-103, 1988

Ninomiya H., Taniguchi T., Fujiwara M. and Kameyama M.
Increased binding of [³H]muscimol and [³H]flunitrazepam in the rat brain under hypoxia.
J. Neurochem. 51, 1111-1117, 1988


その他

Kitamura Y. and Ninomiya H.
Smad expression of hepatic satellite cells in liver cirrhosis in vivo and hepatic satellite cell lines in vitro.
Pathol. Int. 53, 18-26, 2003

Kaji R., McCormic F., Kameyama M. and Ninomiya H.
Brainstem auditory evoked potentials in early diagnosis of basilar artery occlusion.
Neurology 35, 240-243, 1985


和　文　原　著

濱谷 美緒　他
Savant症候群が特徴的であった成人発症のニーマン・ピック病C型 variant biochemical phenotypeの一例
臨床神経学, 56, 422-429, 2016

二宮 治明、中村 慎一、梶 龍児、山尾 哲、亀山 正邦
Startle disease の一例
臨床神経学, 24, 778-781, 1984


著　書

二宮 治明
N教授の生理学講義ノート、 日本医事新報社、2016

仲宗根眞恵、二宮 治明
神経変性の背景
ニーマン・ピック病C型の診断と治療 p36-38、 医薬ジャーナル、 2015

二宮治明
神経系
標準臨床検査学 基礎医学 人体の構造と機能 p122-161、 医学書院、 2015

大野 耕策、二宮 治明
神経回路網形成障害を中心とした脳発達障害研究の進歩
脳科学研究の現状と課題 p187-200、じほう出版、2003

二宮 治明
脳と神経
分子生物学への招待、三共出版、2002

亀山 正邦、二宮 治明、山尾 哲
びっくり病
神経疾患の診かた p412-419 医学書院 1988


英　文　レビュー

Higaki K., Ninomiya H., Suzuki Y. and Nanba E.
Candidate molecules for chemical chaperone therapy of GM1-gangliosidosis.
Future Med. Chem. 5, 1551-1558, 2013

Ninomiya H.
Niemann-Pick C1-Like1: a key player in intestinal cholesterol transport.
Yonago Acta Medica 53, 25-32, 2010

Ninomiya H
Intracellular cholesterol transport by NPC1/NPC2: mysteries of Niemann-Pick disease type C.
Functional and structural biology on the lipo-network
Research Signpost, pp1-15, 2007

Ohno K., Yakura N., Zhang H., Ninomiya H., and Nanba E.
Molecular epidemiology of tubelous sclerosis.
Gann monograph on cancer research 46, 53-59, 2000

Masaki T., Ninomiya H., Sakamoto A. and Okamoto Y.
Structural basis of the function of endothelin receptor.
Mol. Cell. Biochem. 190, 153-156, 1999

Masaki T., Miwa S., Sawamura T., Ninomiya H. and Okamoto Y.
Subcellular mechanisms of endothelin action in vascular system.
Eur. J. Pharmacol. 375, 133-138, 1999

Okamoto Y., Ninomiya H. and Masaki T.
Posttranslational modifications of endothelin receptor type B.
Trends Cardiovasc. Med. 8, 327-329, 1998

Roch J.M., Jin L.W., Ninomiya H., Schubert D. and Saitoh T.
Biologically active domain of the secreted form of the amyloid beta/A4 protein precursor.
Ann. NY. Acad. Sci. 695, 149-157, 1993

Ninomiya H., Taniguchi T. and Fujiwara M.
Effects of oxygen depletion on phosphoinositide breakdown in rat brain slices.
Adv. Exp. Med. Biol. 287, 125-139, 1991

Ninomiya H., Taniguchi T. and Fujiwara M.
Effects of hypoxia on muscarinic cholinergic and benzodiazepine/gamma-aminobutyric acid receptors in the rat brain.
Adv. Exp. Med. Biol. 236, 61-69, 1988


Proceedings

Okamoto Y., Ninomiya H., Tanioka M., Sakamoto A., Miwa S. and Masaki T.
Cysteine residues in the carboxyl terminal domain of the endothelin-B receptor are required for coupling with G-protein.
J. Cardiovasc. Pharmacol. 31, S230-232, 1998

Enoki T., Miwa S., Sakamoto A., Minowa T., Komuro T., Kobayashi S., Ninomiya H. and Masaki T.
Functional coupling of ETA receptor with Ca²⁺-permeable nonselective cation channel in mouse fibroblasts and rabbit aortic smooth-muscle cells.
J. Cardiovasc. Pharmacol. 26, S258-261, 1995

Ninomiya H., Takagi Y., Miwa S. and Masaki T.
Distinct roles of second and third intracellular loops of human endothelin receptors in the selective activation of G.
J. Cardiovasc. Pharmacol. 26, S254-257, 1995

Shimohama S., Ninomiya H., Saitoh T., Terry R.D., et al.
Changes in signal transduction in Alzheimer's disease.
J. Neural Transm. 30, S69-78, 1990


邦　文　レビュー

仲宗根 眞恵、二宮 治明
Niemann-Pick 病 C 型の分子病態機序
神経内科　80, 625-631, 2014

二宮治明
今、明らかにされるNPC1/NPC1-L1の機能
Cardio-Lipidology　4, 16-21, 2010

.二宮治明
腸管のコレステロール吸収におけるNPC1-Like1の役割
消化と吸収　31, 133-139, 2009

二宮治明
ニーマン・ピック C1
生体の科学 56, 458-459, 2005

二宮治明
NPC1/HE1 による細胞内コレステロール輸送
生化学 76, 571-577, 2004

二宮治明
ニーマン・ピック病型での神経細胞死
Clinical Neuroscience 21, 146-147, 2003

大野耕策、二宮治明
ニーマン・ピック病C型の分子病態
遺伝子医学 7, 86-92, 2003

大野耕策、二宮治明
ニーマン・ピック病Ｃ型の分子生物学と治療的展望
日本マス・スクリーニング学会誌 12, 35-42, 2002

谷口美也子、二宮治明、大野耕策
C型Niemann-Pick病
日本臨床 59, 309-31, 2001

二宮治明、大野耕策
ニーマンピック病C型とコレステロール
蛋白質核酸酵素 44, 1213-1218, 1999

二宮治明
グリシンによる抑制性シナプス伝達の欠損
Clinical Neuroscience 14, 950-951, 1996

二宮治明
Startle disease.
Clinical Neuroscience 13, 1318-1319, 1995

二宮治明、眞崎知生
エンドセリンと内皮.
細胞工学 14, 403-408, 1994

二宮治明
アミロイド/A4 前駆体蛋白質の生理機能と血管
血管 16, 199-205, 1993

二宮治明
エンドセリン受容体の構造活性連関
医学のあゆみ 168, 237-240, 1993


招　待　講　演

二宮治明
NPC1/NPC2の構造と機能/神経遺伝病治療戦略セミナー
第55回日本小児神経学会学術集会、大分、2013

Ninomiya H.
Cholesterol transport mediated by NPC1/NPC1-L1
日本生化学会シンポジウム、京都、2011

二宮治明
コレステロール輸送のエスコート蛋白質NPC1/NPC1-Like1
食後代謝懇話会、東京、2009

Ninomiya H.
Proteasomal degradation of NPC1.
Annual Scientific Conference on NPC, Tucson, U.S.A., 2007

二宮治明
ニーマン・ピック病C型細胞での脂質輸送障害とシグナル伝達の変化
形態科学シンポジウム、名古屋、2005

二宮治明
細胞内脂質輸送の障害と神経細胞の障害修復機構
日本生化学会中部支部例会シンポジウム、名古屋、2005

二宮治明
Niemann-Pick病C型:細胞内コレステロール輸送の破綻による神経細胞死
京滋神経セミナー、京都、2005

二宮治明
Niemann-Pick病と脂質ドメイン・ソーテイング
特定領域研究「スフィンゴ脂質による生体膜ドメイン形成と多機能シグナリング」公開シンポジウム、 東京、2003

二宮治明
Niemann-Pick病C型での神経細胞死
若手研究者のための薬理学セミナー、京都、2002

二宮治明
Niemann-Pick病C型での神経細胞死
日本生化学会シンポジウム、京都、2002

二宮治明
エンドセリン受容体を介する情報伝達機構
大阪大学蛋白質研究所セミナー「血管作動性ペプチド研究の新展開」、吹田、1997

Ninomiya H., Okamoto Y., Sakamoto A. and Masaki T.
Structure and function of endothelin receptor.
Peptide receptors, Montreal, Canada, 1996

Ninomiya H., Takagi Y., Miwa S. and Masaki T.
Structural basis of G protein specificity of human ETRs: a study with ETA/B chimeras.
Fourth international conference on endothelin, London, UK, 1996

Ninomiya H., Enoki T., Kobayashi S., Miwa S. and Masaki T.
Versatile intracellular signal transduction through endothelin receptors.
日本分子生物学会シンポジウム、幕張メッセ、1994