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(In Japanese)オルガネラ構築と細胞機能発現制御の分子機構

Research report code R000000118
Posted date Sep 30, 2002
Researchers
  • (In Japanese)藤木 幸夫
Affiliation
  • (In Japanese)九州大学大学院理学系研究院生物科学部門
Research organization
  • (In Japanese)九州大学大学院理学系研究院生物科学部門
Report name (In Japanese)オルガネラ構築と細胞機能発現制御の分子機構
Technology summary (In Japanese)ペルオキシソーム,核,ミトコンドリア,小胞体,ゴルジ体,リソソームなど細胞内オルガネラの動的存在状態とその制御機構,並びに種々の病態をもたらす障害機構をペルオキシソーム系を主体として明らかにし,それらを総合的に考察することにより「オルガネラ構築と細胞機能発現制御」の基本原理を導き出すことを目的として,in vivo,in vitroの両系を駆使して蛋白質の細胞内選別輸送・局在化およびペルオキシソーム系を中心にオルガネラ形成にかかわるより多くの細胞内因子の同定とそれらの機能解明をおこなった。本課題研究は遺伝情報発現ステップの全貌の理解という生命科学研究における極めて重要な命題解明に分子的基盤を与えるだけでなく,その成果として,将来有用物質の分泌生産,オルガネラを利用したバイオテクノロジーや人工細胞の開発などの応用への道も開くことが期待される。
Research field
  • Proteins and peptides in general
  • Cell physiology in general
  • Cell components in general
  • Function of cell components
Published papers related (In Japanese)(1)Toyama, R., Mukai, S., Itagaki, A., Tamura, S., Shimozawa, N., Suzuki, Y., Kondo, N., Wanders, R.J.A., and Fujiki, Y.: Isolation, characterization, and mutation analysis of PEX13-defective Chinese hamster ovary cell mutants. Hum. Mol. Genet. 8: 1673-1681 (1999).
(2)Matsuzono, Y., Kinoshita, N., Tamura, S., Shimozawa, N., Hamasaki, M., Ghaedi, K., Wanders, R.J.A., Suzuki, Y., Kondo, N., and Fujiki, Y.: Human PEX19: cDNA cloning by functional complementation, mutation analysis in a patient with Zellweger syndrome and potential role in peroxisomal membrane assembly. Proc. Natl. Acad. Sci. USA. 96: 2116-2121 (1999).
(3)Zhang, Z., Suzuki, Y., Shimozawa, N., Fukuda, S., Imamura, A., Tsukamoto, T., Osumi, T., Fujiki, Y., Orii, T., Wanders, R.J.A., Barth, P.G., Moser, H.W., Paton, B.C., Besley, G.T., and Kondo, N.: Geneomic structure and identification of 11 novel mutations of PEX6 (peroxisome assembly factor-2) gene in patients with peroxisome biogenesis disorders. Hum. Mut. 13: 487-496 (1999).
(4)Shimozawa, N., Suzuki, Y., Zhang, Z., Imamura, A., Toyama, R., Mukai, S., Fujiki, Y., Tsukamoto, T., Osumi, T., Orii, T., Wanders, R.J.A., and Kondo, N.: Nonsense and temperature-sensitive mutations in PEX13 are the cause of complementation group H of peroxisome biogenesis disorders. Hum. Mol. Genet. 8: 1077-1083 (1999).
(5)Harano, T., Shimizu, N., Otera, H., and Fujiki, Y.: Transmembrane topology of the peroxin Pex2p, an essential component of peroxisome assembly. J. Biochem. 125: 1168-1174 (1999).
(6)Ghaedi, K., Itagaki, A., Toyama, R., Tamura, S., Matsumura, T., Kawai, A., Shimozawa, N., Suzuki, Y., Kondo, N., and Fujiki, Y.: Newly identified Chinese hamster ovary cell mutants defective in peroxisome assembly represent complementation group A of human peroxisome biogenesis disorders and one novel group in mammals. Exp. Cell Res. 248: 482-488 (1999).
(7)Ghaedi, K., Kawai, A., Okumoto, K., Tamura, S., Shimozawa, N., Suzuki, Y., Kondo, N., and Fujiki, Y.: Isolation and characterization of novel peroxisome biogenesis-defective Chinese hamster ovary cell mutants using green fluorescent protein. Exp. Cell Res. 248: 489-497 (1999).
(8)Shimizu, N., Itoh, R., Hirono, H., Otera, H., Ghaedi, K., Tateishi, K., Tamura, S., Okumoto, K., Harano, T., Mukai, S., and Fujiki, Y.: The Peroxin Pex14p: cDNA cloning by functional complementation on a Chinese hamster ovary cell mutant, characterization, and functional analysis. J. Biol. Chem. 274: 12593-12604 (1999).
(9)Saito, M., Iwamori, M., Lin, Bei, Oka, A., Fujiki, Y., Shimozawa, N., Kamoshita, S., Yanagisawa, M., Sakakihara, Y.: Accumulation of glycolipids in mutant Chinese hamster ovary cells (Z65) with defective peroxisomal assembly and comparison of the metabolic rate of glycoshingolipids between Z65 cells and wild-type CHO-K1 cells. Biochim. Biophys. Acta 1438: 55-62 (1999).
(10)Shimozawa, N., Zhang, Z., Suzuki, Y., Imamura, A., Tsukamoto, T., Osumi, T., Fujiki, Y., Orii, T., Barth, P.G., Wanders, R.J.A., and Kondo, N.: Functional heterogeneity of C-terminal peroxisome targeting signal 1 in PEX5-defective patients. Biochem. Biophys. Res. Commun. 262: 504-508 (1999).
(11)Shimozawa, N., Imamura, A., Zhang, Z., Suzuki, Y., Orii, T., Tsukamoto, T., Osumi, T., Fujiki, Y., Wanders, R.J.A., Besley, G., and Kondo, N.: Defective PEX gene products correlate with the protein import, biochemical abnormalities, and phenotypic heterogeneity in peroxisome biogenesis disorders. J. Med. Genet. 36: 779-781 (1999).
(12)Shimozawa, N., Zhang, Z., Imamura, A., Suzuki, Y., Fujiki, Y., Tsukamoto, T., Osumi, T., Aubourg, P., Wanders, R.J.A., and Kondo, N.: Molecular mechanism of detectable catalase-containing particles, peroxisomes in fibroblasts from patients with peroxisome biogenesis disorders. Biochem. Biophys. Res. Commun. 268: 31-35 (2000).
(13)Imamura, A., Shimozawa, N., Suzuki, Y., Zhang, Z., Tsukamoto, T., Fujiki, Y. Orii, T., Osumi, T., and Kondo, N.: Restoration of biochemical function of peroxisome in the temperature-sensitive mild forms of peroxisome biogenesis disorder in humans. Brain & Development 22: 8-12 (2000).
(14)藤木幸夫:ペルオキシソーム形成不全症.増大特集「病気の細胞分子生物学」生体の科学 50:461-463 (1999).
Research project
  • Core Research for Evolutional Science and Technology;Genetic Programming
Information research report
  • (In Japanese)藤木 幸夫. 生命活動のプログラム オルガネラ構築と細胞機能発現制御の分子機構. 戦略的基礎研究推進事業 平成11年度 研究年報.科学技術振興事業団, 2000. p.46 - 57.

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