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Cholesterol-dependent regulation of intracellular localization of NPC1-L1

Research Project

Project/Area Number 20590281
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field General medical chemistry
Research InstitutionTottori University

Principal Investigator

NINOMIYA Haruaki  Tottori University, 医学部, 教授 (80212124)

Co-Investigator(Renkei-kenkyūsha) HIGAKI Katumi  鳥取大学, 生命機能研究支援センター, 准教授 (90294321)
Project Period (FY) 2008 – 2010
Project Status Completed (Fiscal Year 2010)
Budget Amount *help
¥4,810,000 (Direct Cost: ¥3,700,000、Indirect Cost: ¥1,110,000)
Fiscal Year 2010: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2009: ¥1,430,000 (Direct Cost: ¥1,100,000、Indirect Cost: ¥330,000)
Fiscal Year 2008: ¥1,950,000 (Direct Cost: ¥1,500,000、Indirect Cost: ¥450,000)
Keywordsニーマンピック / コレステロール / Rab / NPC1-L1 / トランスポート / ユビキチン
Research Abstract

By using transient gene expression system in HEK293 cells, we identified Rab proteins that participate in cholesterol-dependent intracellular sorting of Niemann-Pick C1-Like1 (NPC1-L1) protein. We also studied intracellular localization of endogenous NPC1-L1 in HepG2 cells and found that localization of the endogenous NPC1-L1 was also regulated in a cholesterol-dependent manner.

Report

(4 results)
  • 2010 Annual Research Report   Final Research Report ( PDF )
  • 2009 Annual Research Report
  • 2008 Annual Research Report
  • Research Products

    (27 results)

All 2011 2010 2009 2008 Other

All Journal Article (23 results) (of which Peer Reviewed: 23 results) Remarks (4 results)

  • [Journal Article] Lysosomal accumulation of Trk protein in brain of GM1-gangliosidosis mouse and its restoration by chemical chaperone.2011

    • Author(s)
      Takamura A., Higaki K., Ninomiya H., Takai T., Matsuda J., Iida M., Ohno K., Suzuki Y., Nanba E.
    • Journal Title

      J.Neurochem. in press

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Chemical chaperone therapy : chaperone effect on mutant enzyme and cellular pathophysiology in β-galactosidase deficiency.2011

    • Author(s)
      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., Nanba E.
    • Journal Title

      Human Mut. in press

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Transcriptional activation of the anchoring protein SAP97 by heat shock factor (HSF)-1 stabilizes K(v) 1.5 channels in HL-1 cells.2011

    • Author(s)
      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., Hisatome I.
    • Journal Title

      Br.J.Pharmacol. in press

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Copper incorporation into ceruloplasmin is regulated by Niemann-Pick C1 protein.2011

    • Author(s)
      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., Sata M.
    • Journal Title

      Hepatol Res. 41

      Pages: 484-491

    • NAID

      10031144114

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Reciprocal control of hERG stability by Hsp70 and Hsc70 with implication for restoration of LQT2 mutant stability.2011

    • Author(s)
      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., Hisatome I.
    • Journal Title

      Cir.Res. 108

      Pages: 458-468

    • NAID

      130002130408

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Chemical chaperone therapy : luciferase assay for screening of β-galactosidase mutations.2010

    • Author(s)
      Li L., Higaki K., Ninomiya H., Luan Z., Iida M., Ogawa S., Suzuki Y., Ohno K., Nanba E.
    • Journal Title

      Mol.Genet.Metab. 101

      Pages: 364-369

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] A fluorescent sp^2-iminosugar with pharmacological chaperone activity for Gaucher disease : synthesis and intracellular distribution studies.2010

    • Author(s)
      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., Suzuki Y.
    • Journal Title

      Chembiochem. 11

      Pages: 2453-2464

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Different distribution of Cav3.2 and Cav3.1 transcripts encoding T-type Ca(2+) channels in the embryonic heart of mice.2010

    • Author(s)
      Mizuta E., Shirai M., Arakawa K., Hidaka K., Miake J., Ninomiya H., Kato M., Shigemasa C., Shirayoshi Y., Hisatome I., Morisaki T.
    • Journal Title

      Biomed.Res. 31

      Pages: 301-305

    • NAID

      130004903809

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] The effect of N-octyl-β-valienamine on β-glucosidase activity in tissues of normal mice.2010

    • Author(s)
      Luan Z., Ninomiya H., Ohno K., Ogawa S., Kubo T., Iida M., Suzuki Y.
    • Journal Title

      Brain Dev. 32

      Pages: 805-809

    • NAID

      10029559648

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] The pharmacological chaperone effect of N-octyl-β-valienamine on human mutant acid β-glucosidases.2010

    • Author(s)
      Luan Z., Li L., Ninomiya H., Ohno K., Ogawa S., Kubo T., Iida M., Suzuki Y.
    • Journal Title

      Blood Cells Mol.Diseases 44

      Pages: 48-54

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Effect of losartan and benzbromarone on the level of human urate transporter 1 mRNA.2010

    • Author(s)
      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., Hisatome I.
    • Journal Title

      Arzneimittelforschung 60

      Pages: 186-188

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Effects of a low-dose antihypertensive diuretic in combination with losartan, telmisartan, or candesartan on serum urate levels in hypertensive patients.2010

    • Author(s)
      Hamada T., Mizuta E., Kondo T., Hirai M., Yamada K., Kato M., Shigemasa C., Yamamoto Y., Ninomiya H., Igawa O., Hisatome I.
    • Journal Title

      Arzneimittelforschung 60

      Pages: 71-75

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Chemical chaperone therapy : luciferase assay for screening of β-galactosidase mutations2010

    • Author(s)
      Li, et al.
    • Journal Title

      Mol.Genet.Metab.

      Volume: 101 Pages: 364-369

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] The effect of N-octyl-β-valienamine on β-glucosidase activity in tissues of normal mice.2010

    • Author(s)
      Luan, et al.
    • Journal Title

      Brain Dev.

      Volume: 32 Pages: 805-809

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] The pharmacological chaperone effect of N-octyl-β-valienamine on human mutant acid β-glucosidases.2010

    • Author(s)
      Luan, et al.
    • Journal Title

      Blood Cells Mol.Diseases

      Volume: 44 Pages: 48-54

    • Related Report
      2010 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Chaperone activity of bicyclic nojirimycin analogues for Gaucher mutations in comparison with N-(n-nonyl)deoxynojirimycin.2009

    • Author(s)
      Luan Z., Higaki K., Aguilar-Moncayo M., Ninomiya H., Ohno K., Garcia-Moreno M.I., Ortiz M.C., Garcia Fernandez J.M., Suzuki Y.
    • Journal Title

      Chembiochem. 23

      Pages: 2780-2792

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Niemann-Pick C1 protein transports copper to the secretory compartment from late endosomes where ATP7B resides.2009

    • Author(s)
      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., Sata M.
    • Journal Title

      Exp.Cell Res. 315

      Pages: 119-126

    • Related Report
      2010 Final Research Report
    • Peer Reviewed
  • [Journal Article] Niemann-Pick C1 protein transports copper to the secretory compartment from late endosomes where ATP7B resides2009

    • Author(s)
      Yanagimoto, et al.
    • Journal Title

      Exp.Cell Res. 315

      Pages: 119-126

    • Related Report
      2009 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Stabilizing effects of eicosapentaenoic acid on Kvl.5 channel protein expressed in mammalian cells.2009

    • Author(s)
      Koshida, et al.
    • Journal Title

      Eur.J.Pharmacol. 604

      Pages: 93-102

    • Related Report
      2009 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Chaperone activity of bicyclic nojirimycin analogues for Gaucher mutations in comparison with N-(n-nonyl)deoxynojirimycin.2009

    • Author(s)
      Luan, et al.
    • Journal Title

      Chembiochem. 23

      Pages: 2780-2792

    • Related Report
      2009 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Brainstem neuropathology in a mouse model of Niemann-Pick disease type C.2008

    • Author(s)
      Luan, et al.
    • Journal Title

      J. Neurol. Sci. 268

      Pages: 108-116

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Enhanced autophagy and mitochondrial aberrations in murine GM1-gagliosidosis.2008

    • Author(s)
      Takamura, et al.
    • Journal Title

      Biochem. Biophys. Res. Commun. 367

      Pages: 616-622

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Journal Article] Ubiquitin-proteasome system impairment caused by a missense cardiac myosin-binding protein C mutation and associated with cardiac dysfunction in hypertrophic cardiomyopathy.2008

    • Author(s)
      Bahrudin, et al.
    • Journal Title

      J Mol. Biol. 384

      Pages: 896-907

    • Related Report
      2008 Annual Research Report
    • Peer Reviewed
  • [Remarks] ホームページ等

    • URL

      http://www.ninomiya.med.tottori-u.ac.jp/homepage/

    • Related Report
      2010 Final Research Report
  • [Remarks]

    • URL

      http://www.ninomiya.med.tottori-u.ac.jp/homepage/

    • Related Report
      2010 Annual Research Report
  • [Remarks]

    • URL

      http://www.ninomiya.med.tottori-u.ac.jp/homepage/

    • Related Report
      2009 Annual Research Report
  • [Remarks]

    • URL

      http://www.asahi-net.or.jp/~ev6h-nnmy/

    • Related Report
      2008 Annual Research Report

URL: 

Published: 2008-04-01   Modified: 2016-04-21  

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