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Peroxisome biogenesis and human eroxisome assembly disorders.

Research Project

Project/Area Number 07408016
Research Category

Grant-in-Aid for Scientific Research (A)

Allocation TypeSingle-year Grants
Section一般
Research Field Functional biochemistry
Research InstitutionKYUSHU UNIVERSITY

Principal Investigator

FUJIKI Yukio  KYUSHU UNIVERSITY Faculty of Science, Professor, 理学部, 教授 (70261237)

Co-Investigator(Kenkyū-buntansha) TAMURA Shigehiko  KYUSHU UNIVERSITY Faculty of Science, Research associate, 理学部, 助手 (90236753)
HARANO Tomoyuki  KYUSHU UNIVERSITY Faculty of Science, Research associate, 理学部, 助手 (80037275)
Project Period (FY) 1995 – 1997
Project Status Completed (Fiscal Year 1997)
Budget Amount *help
¥30,800,000 (Direct Cost: ¥30,800,000)
Fiscal Year 1997: ¥7,400,000 (Direct Cost: ¥7,400,000)
Fiscal Year 1996: ¥8,100,000 (Direct Cost: ¥8,100,000)
Fiscal Year 1995: ¥15,300,000 (Direct Cost: ¥15,300,000)
Keywordsperoxisome / peroxisome-deficient disorders / CHO cell mutants / peroxin / patient analysis / membrane protein / AAA family / RING finger / 蛋白質-蛋白質間相互作用 / 亜鉛イオン / 部位変異体 / 形成機構 / Zellweger症候群 / 相補性解析 / 相補遺伝子 / 局在化シグナル
Research Abstract

I.Isolation, characterization, and complementation group analysis of novel CHO cell mutants defective in peroxisome biogenesis.
In addition to previously isolated, three complementation groups (CG_S) of peroxisome-deficient CHO cell mutants, ZP24, Z65, and ZP92, we isolated ZP107 (the same group as Z24), ZP105/ZP139, ZP109, ZP110.ZP114, and ZP119, by the P9OH/UV method. CG analysis by PEX cDNA transfection and/or cell fusion with previously identified CGs of mutant cells, including 10 groups of fibroblasts derived from patients with peroxisomal disorders, revealed ZP110, ZP114, and ZP119 to be in 3 novel CGs. Thus, it is evident that peroxisome assembly requires at least 13 genes products.
II.Cloning of PEX12 and PEX1
By genetic functional complementation assay, PEX12 and PEX1 were cloned for ZP109 and ZP107, respectively. Mutation analysis of PEX12 and PEX1 in patients with Zellweger syndorome of CGs III and I,respectively, were also done. Inactivation of PEX12 and PEX1 was demonstrated to be the genetic cause of CGs III and I peroxisome deficiency disorders, respectively. Moreover, we found Pex5p (PTS1 receptor) to be involved in import of not only PTS1 but also PTS2 protein, using CGII ZP105 and ZP139 of complementation group III.

Report

(4 results)
  • 1997 Annual Research Report   Final Research Report Summary
  • 1996 Annual Research Report
  • 1995 Annual Research Report
  • Research Products

    (33 results)

All Other

All Publications (33 results)

  • [Publications] Tamura, S.: "Human PEX1 cloned by functional complementation on a CHO cell mutant is responsible for peroxisome-deficient Zellweger syndrome of complementation group I." Proc.Natl.Acad.Sci.USA. 95(in press). (1998)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Otera, H.: "Peroxisome targeting signal type 1(PTS-1)-receptor is involved in import of both PTS-1and PTS-2protein:studies with PEX5-defective CHO cell mutants." Mol.Cell.Biol.18. 388-399 (1998)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Okumoto, K.: "PEX12 encodes an integral membrane protein of peroxisomes." Nature Genet.17. 265-266 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Fujiki, Y.: "Molecular defects in genetic disease of peroxisomes." Biochim.Biophys.Acta. 1361. 235-250 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Tateishi, K.: "Newly identified Chinese hamster ovary (CHO)cell mutatnts defective in peroxisome biogenesis represent two novel complementation groups in mammals." Eur.J.Cell Biol.73. 352-359 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Okumoto, K.: "Isolation and characterization of peroxisome-deficient Chinese hamster ovary (CHO)cell mutants representing human complementation group III." Exp.Cell Res.233. 11-20 (1997)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Tamura, S.: "Human PEX1 cloned by functional complementation on a CHO cell mutant is responsible for peroxisome-deficient Zellweger syndrome of complementation group I." Proc.Natl.Acad.Sci.USA. 95 : (inpress). (1998)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Otera, H.: "Peroxisome targeting signal type 1 (PTS-1)-receptor is involved in import of both PTS-1 and PTS-2protein : Studies with PEX5-defective CHO cell mutants." Mol.Cell.Biol.18. 388-399 (1998)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Okumoto, K.: "PEX12 encodes an integral membrane protein of peroxisomes." Nature Genet.17. 265-266 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Fujiki, Y.: "Molecular defects in genetic disease of peroxisomes." Biochim.Biophys.Acta. 1361. 235-250 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Tateishi, K.: "Newly identified Chinese hamster ovary (CHO) cell mutants defective in peroxisome biogenesis represent two novel complementation groups in mammals." Eur.J.Cell Biol.73. 352-359 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Okumoto, K.: "Isolation and characterization of peroxisome-deficient Chinese hamster ovary (CHO) cell mutants representing human complementation group III." Exp.Cell Res.233. 11-20 (1997)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      1997 Final Research Report Summary
  • [Publications] Tamura,S.: "Human PEX1 cloned by functional complementation on a CHO cell mutant is responsible for peroxisome-deficient Zellweger syndrome of complementation group I." Proc.Natl.Acad.Sci.USA. 95. in press (1998)

    • Related Report
      1997 Annual Research Report
  • [Publications] Otera,T.: "Peroxisome targeting signal type 1(PTS-1)-receptor is involved in import of both PTS-1 and PTS-2protein:Studies with PEX5-defective CHO cell mutants." Mol.Cell.Biol.18. 388-399 (1998)

    • Related Report
      1997 Annual Research Report
  • [Publications] Okumoto,K.: "PEX12 encodes an integral membrane protein of peroxisomes." Nature Genet.17. 265-266 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] Fujiki,Y.: "Molecular defects in genetic disease of peroxisomes." Biochim.Biophys.Acta. 1361. 235-250 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] Tateishi,K.: "Newly identified Chinese hamster ovary(CHO)cell mutants defective in peroxisome biogenesis represent two novel complementation groups in mammals." Eur.J.Cell Biol.73. 352-359 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] Okumoto,K.: "Isolation and characterization of peroxisome-deficient Chinese hamster ovary(CHO)cell mutants representing human complementation group III." Exp.Cell Res.233. 11-20 (1997)

    • Related Report
      1997 Annual Research Report
  • [Publications] Shimozawa,N.: "A novel mutation,R125X in peroxisome assembly factor-1 responsible for Zellweger syndrome." Hum.Mut.(in press). (1997)

    • Related Report
      1996 Annual Research Report
  • [Publications] Tsukamoto,T.: "Isolation of a new peroxisome deficient CHO cell mutant defective in peroxisome targeting signal-1 receptor." Biochem.Biophys.230. 402-406 (1997)

    • Related Report
      1996 Annual Research Report
  • [Publications] Fujiki,Y.: "Approaches to Studies on Peroxisome Biogenesis and Human Peroxisome-deficient Disorders." Ann.N.Y.Acad.Sci.804. 491-501 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Shimozawa,N.: "Correction by Gene Expression of Biochemical Abnormalities in Fibroblast from Zellweger Synreome.Pediatr." Pediatr.Res.39. 812-815 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Fukuda,S.: "Human peroxisome assembly factor-2(PAF-2):A gene responsible for group C peroxisome biogenesis disorder in humans." Am.J.Hum.Genet.59. 1210-1220 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] 藤木幸夫: "特集によせて--特集「酸素毒」" 組織培養. 22. 123-124 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Fujiki,Y.: "In Membrane Proteins:Structure,Function and Expression Control(Mihara,K.and Hamasaki,N.des.)" Karger Press, 53-61 (1997)

    • Related Report
      1996 Annual Research Report
  • [Publications] Fujiki,Y.: "Peroxisome Biogenesis:Topogenic Signal,Peroxisome Assembly Factor,and Zellweger Syndrome.In Membrane Protein Transport(S.Rothman,ed.)" Advances in Molecular and Cell Biology, 213-219 (1996)

    • Related Report
      1996 Annual Research Report
  • [Publications] Fujiki, Y.: "Approaches to Studies on Peroxisome Biogenesis and Human Peroxisome-deficient Disorders." Ann. N. Y. Acad. Sci.(in press). (1996)

    • Related Report
      1995 Annual Research Report
  • [Publications] Shimozawa, N.: "Correction by Gene Expression of Biochemical Abnormalities in Fibroblasts from Zellweger Syndrome. Pediatr." Pediatr. Res.(in press). (1996)

    • Related Report
      1995 Annual Research Report
  • [Publications] Tsukamoto, T.: "Peroxisome Assembly Factor-2: A Putative ATPase Cloned by Functional Complementation on a Peroxisome-dificient Mammalian Cell Mutant." Nature Genet.11. 395-401 (1995)

    • Related Report
      1995 Annual Research Report
  • [Publications] Nakai, T.: "Multiple Genes, Including a Member of the AAA Family, are Essential for Degradation of Unassembled Subunit 2 of Cytochrome c Oxidase in Yeast Mitochondoria." Mol. Cell. Biol.15. 4441-4452 (1995)

    • Related Report
      1995 Annual Research Report
  • [Publications] 藤木幸夫: "ペルオキシソーム病の遺伝子診断「遺伝子診断-その現状と展望」" 医学のあゆみ. 174. 477-483 (1995)

    • Related Report
      1995 Annual Research Report
  • [Publications] 藤木幸夫: "総説:ペルオキシソームの形成機構とペルオキシソーム病" 生化学. 67. 204-223 (1995)

    • Related Report
      1995 Annual Research Report
  • [Publications] Fujiki, Y.: "Peroxisome Biogenesis: Topogenic Signal, Peroxisome Assembly Factor, and Zellweger Syndrome. In Membrane Protein Transport(S. Rothman, ed)" Advances in Molecular and Cell Biology (in press), (1995)

    • Related Report
      1995 Annual Research Report

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Published: 1995-04-01   Modified: 2016-04-21  

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