Yukio Fujiki

Summary

Affiliation: Kyushu University
Country: Japan

Publications

  1. ncbi Peroxisome biogenesis and molecular defects in peroxisome assembly disorders
    Y Fujiki
    Department of Biology, Graduate School of Science, Kyushu University, Fukuoka 812 8581, Japan
    Cell Biochem Biophys 32:155-64. 2000
  2. pmc The peroxisomal membrane protein import receptor Pex3p is directly transported to peroxisomes by a novel Pex19p- and Pex16p-dependent pathway
    Takashi Matsuzaki
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    J Cell Biol 183:1275-86. 2008
  3. ncbi Molecular mechanisms of import of peroxisome-targeting signal type 2 (PTS2) proteins by PTS2 receptor Pex7p and PTS1 receptor Pex5pL
    Satoru Mukai
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    J Biol Chem 281:37311-20. 2006
  4. ncbi Lessons from peroxisome-deficient Chinese hamster ovary (CHO) cell mutants
    Yukio Fujiki
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Fukuoka 812 8581, Japan
    Biochim Biophys Acta 1763:1374-81. 2006
  5. ncbi [Peroxisome biogenesis and human peroxisomal disorders]
    Yukio Fujiki
    Tanpakushitsu Kakusan Koso 49:1136-42. 2004
  6. ncbi Import of peroxisomal membrane proteins: the interplay of Pex3p- and Pex19p-mediated interactions
    Yukio Fujiki
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Fukuoka 812 8581, Japan
    Biochim Biophys Acta 1763:1639-46. 2006
  7. doi Dynamic and functional assembly of the AAA peroxins, Pex1p and Pex6p, and their membrane receptor Pex26p involved in shuttling of the PTS1 receptor Pex5p in peroxisome biogenesis
    Yukio Fujiki
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    Biochem Soc Trans 36:109-13. 2008
  8. ncbi Peroxisome biogenesis and peroxisome biogenesis disorders
    Y Fujiki
    Department of Biology, Graduate School of Science, Kyushu University, 6 10 1 Hakozaki, Higashi ku, 812 8581, Fukuoka, Japan
    FEBS Lett 476:42-6. 2000
  9. pmc Tail-anchored PEX26 targets peroxisomes via a PEX19-dependent and TRC40-independent class I pathway
    Yuichi Yagita
    Graduate School of Systems Life Sciences, Faculty of Sciences, Kyushu University, Higashi ku, Fukuoka, Japan
    J Cell Biol 200:651-66. 2013
  10. pmc Mutations in novel peroxin gene PEX26 that cause peroxisome-biogenesis disorders of complementation group 8 provide a genotype-phenotype correlation
    Naomi Matsumoto
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka, Japan
    Am J Hum Genet 73:233-46. 2003

Collaborators

  • T Osumi
  • T Tsukamoto
  • Atsushi Tanaka
  • Kunio Miki
  • Yoshihiro Suzuki
  • Ryo Taguchi
  • N Shimozawa
  • Marc Fransen
  • Kiyoto Motojima
  • Maho Hamasaki
  • Hugo Moser
  • T Mori
  • A Imamura
  • R J Wanders
  • Tomoko Nagase
  • Masao Iwamori
  • Masanori Honsho
  • Non Miyata
  • Kanji Okumoto
  • Shigehiko Tamura
  • S Tamura
  • Satoru Mukai
  • Naomi Matsumoto
  • Yuji Matsuzono
  • Kamran Ghaedi
  • K Okumoto
  • Jian Rong Su
  • N Kondo
  • Takashi Matsuzaki
  • Yuichi Abe
  • Masafumi Noguchi
  • Hidenori Otera
  • Tomoe Umemoto
  • Yuichi Yagita
  • Makiko Saito
  • K Ghaedi
  • H Otera
  • Ann Moser
  • T Harano
  • M Honsho
  • Ryuichi Natsuyama
  • Satomi Furuki
  • Akinori Itoyama
  • I Abe
  • Fumiko Iwamoto
  • Chika Nashiro
  • Kazuki Takeda
  • N Shimizu
  • Yasuhiko Sato
  • Sayuri Hara-Kuge
  • Takahide Hiromasa
  • Shinta Kobayashi
  • N Matsumoto
  • Ryota Itoh
  • Wei Fan
  • Naohiko Kinoshita
  • Yoichi Sakakihara
  • Takashi Igarashi
  • Y Matsuzono
  • Mitsuru Ishizuka
  • R Toyama
  • Noriko Akiyama
  • Eiko Yanago
  • Hiroki Nakanishi
  • Yumi Yoshida
  • Yukari Kametani
  • Yui Matsumoto
  • Sachi Misono
  • Yasuko Hashiguchi
  • Astuko Kashiwagi
  • Shunsuke Asaoku
  • Ken ichiro Hosoi
  • M Nishimura
  • Yuji Kobayashi
  • S Mukai
  • Hiroaki Nakano
  • Tsuneo Imanaka
  • Hiroyuki Shibata
  • Yoshinori Kashiwayama
  • Hiroaki Kato
  • Yuriko Iwamori
  • K Tateishi
  • Makoto Horikawa
  • Masashi Mizuguchi
  • Shinobu Yasutake
  • Shigeki Yuasa
  • Hiroyuki Watanabe
  • Takashi Saito
  • Yoshiro Toyama
  • Arata Takeuchi

Detail Information

Publications72

  1. ncbi Peroxisome biogenesis and molecular defects in peroxisome assembly disorders
    Y Fujiki
    Department of Biology, Graduate School of Science, Kyushu University, Fukuoka 812 8581, Japan
    Cell Biochem Biophys 32:155-64. 2000
    ..Furthermore, we have isolated novel CGs of CHO mutants, ZP119 and ZP126...
  2. pmc The peroxisomal membrane protein import receptor Pex3p is directly transported to peroxisomes by a novel Pex19p- and Pex16p-dependent pathway
    Takashi Matsuzaki
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    J Cell Biol 183:1275-86. 2008
    ..Based on these novel findings, we suggest a model for the import of PMPs that provides new insights into the molecular mechanisms underlying the biogenesis of peroxisomes and its regulation involving Pex3p, Pex19p, and Pex16p...
  3. ncbi Molecular mechanisms of import of peroxisome-targeting signal type 2 (PTS2) proteins by PTS2 receptor Pex7p and PTS1 receptor Pex5pL
    Satoru Mukai
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    J Biol Chem 281:37311-20. 2006
    ..Thus, the Pex7p-mediated PTS2 protein import shares most of the steps with the Pex5p-dependent PTS1 import machinery but is likely distinct at the cargo-releasing stage...
  4. ncbi Lessons from peroxisome-deficient Chinese hamster ovary (CHO) cell mutants
    Yukio Fujiki
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Fukuoka 812 8581, Japan
    Biochim Biophys Acta 1763:1374-81. 2006
    ..Molecular mechanisms of peroxisome assembly are currently investigated by making use of such mammalian cell mutants...
  5. ncbi [Peroxisome biogenesis and human peroxisomal disorders]
    Yukio Fujiki
    Tanpakushitsu Kakusan Koso 49:1136-42. 2004
  6. ncbi Import of peroxisomal membrane proteins: the interplay of Pex3p- and Pex19p-mediated interactions
    Yukio Fujiki
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Fukuoka 812 8581, Japan
    Biochim Biophys Acta 1763:1639-46. 2006
    ..Here we review the recent progress made in our understanding of the role of two key players in PMP biogenesis, Pex3p and Pex19p...
  7. doi Dynamic and functional assembly of the AAA peroxins, Pex1p and Pex6p, and their membrane receptor Pex26p involved in shuttling of the PTS1 receptor Pex5p in peroxisome biogenesis
    Yukio Fujiki
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    Biochem Soc Trans 36:109-13. 2008
    ..AAA peroxins are involved in the export from peroxisomes of Pex5p, the PTS1 (peroxisome-targeting signal type 1) receptor...
  8. ncbi Peroxisome biogenesis and peroxisome biogenesis disorders
    Y Fujiki
    Department of Biology, Graduate School of Science, Kyushu University, 6 10 1 Hakozaki, Higashi ku, 812 8581, Fukuoka, Japan
    FEBS Lett 476:42-6. 2000
    ..Hence, peroxisomes may form de novo and do not have to arise from pre-existing, morphologically recognizable peroxisomes. Impaired peroxisome assembly causes peroxisome biogenesis disorders such as Zellweger syndrome...
  9. pmc Tail-anchored PEX26 targets peroxisomes via a PEX19-dependent and TRC40-independent class I pathway
    Yuichi Yagita
    Graduate School of Systems Life Sciences, Faculty of Sciences, Kyushu University, Higashi ku, Fukuoka, Japan
    J Cell Biol 200:651-66. 2013
    ..Finally, our results suggest that a TMD that escapes capture by TRC40 and is followed by a highly basic luminal domain directs TA proteins to peroxisomes via the PEX19-dependent route...
  10. pmc Mutations in novel peroxin gene PEX26 that cause peroxisome-biogenesis disorders of complementation group 8 provide a genotype-phenotype correlation
    Naomi Matsumoto
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka, Japan
    Am J Hum Genet 73:233-46. 2003
    ..These findings confirm that the degree of temperature sensitivity in pex26 cell lines is predictive of the clinical phenotype in patients with PEX26 deficiency...
  11. doi Cysteine ubiquitination of PTS1 receptor Pex5p regulates Pex5p recycling
    Kanji Okumoto
    Department of Biology, Faculty of Sciences, Kyushu University, Fukuoka 812 8581, Japan
    Traffic 12:1067-83. 2011
    ..The Pex5p-C11A would be a useful tool for gaining a mechanistic insight into the matrix protein import into peroxisomes...
  12. ncbi Functional domains and dynamic assembly of the peroxin Pex14p, the entry site of matrix proteins
    Ryota Itoh
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Higashi ku, Fukuoka 812 8581, Japan
    J Biol Chem 281:10196-205. 2006
    ..Thus, Pex14p most likely forms several distinct peroxin complexes involved in peroxisomal matrix protein import...
  13. ncbi Isolation and characterization of novel phenotype CHO cell mutants defective in peroxisome assembly, using ICR191 as a potent mutagenic agent
    Kamran Ghaedi
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka, Japan
    Cell Biochem Funct 26:684-91. 2008
    ..pex2 ZPEG239 showed a mutation in codon GAG for Glu(201) to a nonsense mutation, TAG. Thus, the method developed here using ICR191 could be useful for isolation of further novel cell mutants impaired in peroxisome biogenesis...
  14. ncbi Mutations in the peroxin Pex26p responsible for peroxisome biogenesis disorders of complementation group 8 impair its stability, peroxisomal localization, and interaction with the Pex1p x Pex6p complex
    Satomi Furuki
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    J Biol Chem 281:1317-23. 2006
    ..Furthermore, we present several lines of evidence that show that the instability, insufficient binding to Pex1p x Pex6p complexes, or mislocalization of patient-derived Pex26p mutants is most likely responsible for the CG8 PBDs...
  15. ncbi Functional domain mapping of peroxin Pex19p: interaction with Pex3p is essential for function and translocation
    Yuji Matsuzono
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Higashi ku, Fukuoka 812 8581, Japan
    J Cell Sci 119:3539-50. 2006
    ..Pex19p probably functions as a chaperone for membrane proteins and transports them to peroxisomes by anchoring to Pex3p using residues 12-73 and 40-131...
  16. ncbi Peroxisome division is impaired in a CHO cell mutant with an inactivating point-mutation in dynamin-like protein 1 gene
    Atsushi Tanaka
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    Exp Cell Res 312:1671-84. 2006
    ..Furthermore, ZP121 was lowered in the level of phospholipids, plasmalogens, and phosphatidylethanolamine and was less sensitive to oxidative stresses. Thus, ZP121 is the first dlp1 mutant in mammalian cells...
  17. ncbi A temperature-sensitive CHO pex1 mutant with a novel mutation in the AAA Walker A1 motif
    Wei Fan
    Graduate School of Systems Life Sciences, Kyushu University, Fukuoka, Japan
    Biochem Biophys Res Commun 345:1434-9. 2006
    ..Together, our findings demonstrate that Walker A1 motif of Pex1p is essential for Pex1p-Pex6p interaction and Pex1p targeting to peroxisomes...
  18. doi AWP1/ZFAND6 functions in Pex5 export by interacting with cys-monoubiquitinated Pex5 and Pex6 AAA ATPase
    Non Miyata
    Department of Biology, Faculty of Sciences, Kyushu University, Fukuoka, Japan
    Traffic 13:168-83. 2012
    ..Taken together, these results identify AWP1 as a novel cofactor of Pex6 involved in the regulation of Pex5 export during peroxisome biogenesis...
  19. ncbi Dynamic and functional assembly of the AAA peroxins, Pex1p and Pex6p, and their membrane receptor Pex26p
    Shigehiko Tamura
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    J Biol Chem 281:27693-704. 2006
    ..These results suggested that Pex1p possesses two distinct oligomeric forms, a homo-oligomer in the cytosol and a hetero-oligomer on peroxisome membranes, possibly playing distinct functions in peroxisome biogenesis...
  20. pmc Shuttling mechanism of peroxisome targeting signal type 1 receptor Pex5: ATP-independent import and ATP-dependent export
    Non Miyata
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Higashi ku, Fukuoka, Japan
    Mol Cell Biol 25:10822-32. 2005
    ..Together, results indicated that Pex5 most likely enters peroxisomes, changes its interacting partners, and then exits using ATP energy...
  21. ncbi The pathogenic peroxin Pex26p recruits the Pex1p-Pex6p AAA ATPase complexes to peroxisomes
    Naomi Matsumoto
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    Nat Cell Biol 5:454-60. 2003
    ..PEX26 expression in ZP167 cells re-establishes colocalization of Pex6 and Pex1 with Pex26, in a Pex6-dependent manner. Thus, Pex26 recruits Pex6-Pex1 complexes to peroxisomes...
  22. ncbi A novel pex2 mutant: catalase-deficient but temperature-sensitive PTS1 and PTS2 import
    Noriko Akiyama
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Higashi ku, Fukuoka 812 8581, Japan
    Biochem Biophys Res Commun 293:1523-9. 2002
    ..Therefore, ZPEG309 is a novel pex2, catalase-deficient mutant with temperature-sensitive PTS1 and PTS2 import...
  23. ncbi Fis1, DLP1, and Pex11p coordinately regulate peroxisome morphogenesis
    Shinta Kobayashi
    Graduate School of Systems Life Sciences, Kyushu University, Fukuoka 812 8581, Japan
    Exp Cell Res 313:1675-86. 2007
    ..Taken together, these findings indicate that Fis1 plays important roles in peroxisome division and maintenance of peroxisome morphology in mammalian cells, possibly in a concerted manner with Pex11pbeta and DLP1...
  24. ncbi The membrane biogenesis peroxin Pex16p. Topogenesis and functional roles in peroxisomal membrane assembly
    Masanori Honsho
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    J Biol Chem 277:44513-24. 2002
    ..These results imply that Pex16p functions in peroxisome membrane assembly, more likely upstream of Pex3p...
  25. doi Pex5p imports folded tetrameric catalase by interaction with Pex13p
    Hidenori Otera
    Department of Biology, Faculty of Sciences, Graduate School of Systems Life Sciences, Kyushu University, Fukuoka, 812 8581, Japan
    Traffic 13:1364-77. 2012
    ..These findings suggest that the Pex5p-Pex13p interaction likely plays a pivotal role in the peroxisomal import of folded and oligomeric proteins...
  26. doi Docosahexaenoic acid mediates peroxisomal elongation, a prerequisite for peroxisome division
    Akinori Itoyama
    Graduate School of Systems Life Sciences, Faculty of Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Higashi ku, Fukuoka 812 8581, Japan
    J Cell Sci 125:589-602. 2012
    ..DHA enhanced peroxisomal division in a microtubule-independent manner. These results suggest that DHA is a crucial signal for peroxisomal elongation, a prerequisite for subsequent fission and peroxisome division...
  27. ncbi In vitro transport of membrane proteins to peroxisomes by shuttling receptor Pex19p
    Yuji Matsuzono
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    J Biol Chem 281:36-42. 2006
    ..Together, these results strongly suggested that Pex19p translocates the membrane peroxins from the cytosol to peroxisomes in an ATP- and Pex3p-dependent manner and then shuttles back to the cytosol...
  28. doi Recruiting mechanism of the AAA peroxins, Pex1p and Pex6p, to Pex26p on the peroxisomal membrane
    Chika Nashiro
    Graduate School of Systems Life Sciences, Kyushu University, Fukuoka 812 8581, Japan
    Traffic 12:774-88. 2011
    ..Taken together, Pex1p and Pex6p are most likely regulated in their peroxisomal localization onto Pex26p via conformational changes by the ATPase cycle...
  29. doi Interaction defect of the medium isoform of PTS1-receptor Pex5p with PTS2-receptor Pex7p abrogates the PTS2 protein import into peroxisomes in mammals
    Masanori Honsho
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Higashi ku, Fukuoka, Japan
    J Biochem 149:203-10. 2011
    ..Together, we confirm the pivotal role of Pex5pL in PTS2 import, showing that the N-terminal 7-amino acid residues in the 37-amino acid insertion of Pex5pL are essential for the binding to Pex7p...
  30. doi In vitro import of peroxisome-targeting signal type 2 (PTS2) receptor Pex7p into peroxisomes
    Non Miyata
    Department of Biology, Faculty of Sciences, Graduate School of Systems Life Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    Biochim Biophys Acta 1793:860-70. 2009
    ....
  31. pmc Crystal structure of the conserved N-terminal domain of the peroxisomal matrix protein import receptor, Pex14p
    Jian Rong Su
    Department of Chemistry, Graduate School of Science, Kyoto University, Sakyo ku, Kyoto 606 8502, Japan
    Proc Natl Acad Sci U S A 106:417-21. 2009
    ..Collectively, Phe-35 and Phe-52 are essential for the Pex14p functions, including the interaction between Pex14p and Pex5p...
  32. pmc Peroxisomal targeting signal receptor Pex5p interacts with cargoes and import machinery components in a spatiotemporally differentiated manner: conserved Pex5p WXXXF/Y motifs are critical for matrix protein import
    Hidenori Otera
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Higashi ku, Fukuoka 812 8581, Japan
    Mol Cell Biol 22:1639-55. 2002
    ..Thus, Pex14p and Pex13p very likely form mutually and temporally distinct subcomplexes involved in peroxisomal matrix protein import...
  33. doi Ligand-dependent nucleo-cytoplasmic shuttling of peroxisome proliferator-activated receptors, PPARα and PPARγ
    Tomoe Umemoto
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Higashi ku, Fukuoka, 812 8581, Japan
    Genes Cells 17:576-96. 2012
    ..Taken together, we suggest that the multiple pathways for the nuclear-cytoplasmic transport of PPARs regulate the biological functions of PPARs in response to external signals...
  34. doi New insights into dynamic and functional assembly of the AAA peroxins, Pex1p and Pex6p, and their membrane receptor Pex26p in shuttling of PTS1-receptor Pex5p during peroxisome biogenesis
    Yukio Fujiki
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    Biochim Biophys Acta 1823:145-9. 2012
    ..Pex5p-C11A is imported into peroxisomes but not exported, hence suggesting an essential role of the cysteine residue in the export of Pex5p...
  35. doi Monomer-dimer transition of the conserved N-terminal domain of the mammalian peroxisomal matrix protein import receptor, Pex14p
    Jian Rong Su
    Department of Chemistry, Graduate School of Science, Kyoto University, Sakyo ku, Kyoto 606 8502, Japan
    Biochem Biophys Res Commun 394:217-21. 2010
    ..The model allows us to discuss the mechanism of the oligomeric transition of the full-length Pex14p modulated by the binding of other peroxins...
  36. ncbi Very-long-chain polyunsaturated fatty acids accumulate in phosphatidylcholine of fibroblasts from patients with Zellweger syndrome and acyl-CoA oxidase1 deficiency
    Yuichi Abe
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    Biochim Biophys Acta 1841:610-9. 2014
    ..Because patients with AOx deficiency suffer from more severe symptoms than those with X-ALD, accumulation of VLC-PUFA and/or reduction of DHA may be associated with the severity of peroxisomal diseases. ..
  37. doi Peroxisome biogenesis disorders: molecular basis for impaired peroxisomal membrane assembly: in metabolic functions and biogenesis of peroxisomes in health and disease
    Yukio Fujiki
    Department of Biology, Kyushu University Graduate School, Fukuoka, Japan
    Biochim Biophys Acta 1822:1337-42. 2012
    ..Another model suggests that in Saccharomyces cerevisiae peroxisomes likely emerge from the endoplasmic reticulum...
  38. doi The peroxin Pex14p is involved in LC3-dependent degradation of mammalian peroxisomes
    Sayuri Hara-Kuge
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Higashi ku, Fukuoka 812 8581, Japan
    Exp Cell Res 314:3531-41. 2008
    ....
  39. doi Isolation and characterization of mutant animal cell line defective in alkyl-dihydroxyacetonephosphate synthase: localization and transport of plasmalogens to post-Golgi compartments
    Masanori Honsho
    Graduate School of Systems Life Sciences, Kyushu University, Fukuoka 812 8581, Japan
    Biochim Biophys Acta 1783:1857-65. 2008
    ....
  40. doi Pex5p stabilizes Pex14p: a study using a newly isolated pex5 CHO cell mutant, ZPEG101
    Ryuichi Natsuyama
    Graduate School of Systems Life Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Higashi ku, Fukuoka 812 8581, Japan
    Biochem J 449:195-207. 2013
    ..Therefore ZPEG101 with no Pex5p would be a useful tool for investigating Pex5p function and delineating the mechanisms underlying peroxisomal matrix protein import...
  41. ncbi Isolation of Chinese hamster ovary cell pex mutants: two PEX7-defective mutants
    Eiko Yanago
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    Biochem Biophys Res Commun 293:225-30. 2002
    ..Therefore, ZPEG227 is a pex7 mutant possessing a newly identified mutation in mammalian pex7 cell lines...
  42. ncbi cDNA cloning and characterization of the third isoform of human peroxin Pex11p
    Atsushi Tanaka
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Higashi ku, Fukuoka 812 8581, Japan
    Biochem Biophys Res Commun 300:819-23. 2003
    ..PEX11gamma was not induced in rats by treatment of clofibrate, a peroxisome proliferator, similar to constitutively expressed PEX11beta but in contrast to inducible PEX11alpha...
  43. pmc Two proteases, trypsin domain-containing 1 (Tysnd1) and peroxisomal lon protease (PsLon), cooperatively regulate fatty acid β-oxidation in peroxisomal matrix
    Kanji Okumoto
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Higashi ku, Fukuoka 812 8581, Japan
    J Biol Chem 286:44367-79. 2011
    ..The proteolytic activity of oligomeric Tysnd1 is in turn controlled by self-cleavage of Tysnd1 and degradation of Tysnd1 cleavage products by PsLon...
  44. ncbi [Peroxisome biogenesis and human disorders]
    Yukio Fujiki
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, 6 10 1, Hakozaki, Higashi ku, Fukuoka 812 8581, Japan
    Seikagaku 75:459-64. 2003
  45. pmc Posttranslational regulation of fatty acyl-CoA reductase 1, Far1, controls ether glycerophospholipid synthesis
    Masanori Honsho
    Department of Biology, Faculty of Sciences, Kyushu University, 6 10 1 Hakozaki, Higashi ku, Fukuoka 812 8581, Japan
    J Biol Chem 285:8537-42. 2010
    ..Taken together, our results indicate that ether lipid biosynthesis in mammalian cells is regulated by a negative feedback mechanism that senses cellular plasmalogen levels and appropriately increases or decreases Far1...
  46. ncbi Intracellular localization, function, and dysfunction of the peroxisome-targeting signal type 2 receptor, Pex7p, in mammalian cells
    Satoru Mukai
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    J Biol Chem 277:9548-61. 2002
    ..Therefore, nearly the full length of Pex7p, including all WD motifs, is required for its function...
  47. doi System to quantify the import of peroxisomal matrix proteins by fluorescence intensity
    Masafumi Noguchi
    Graduate School of Systems Life Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Higashi ku, Fukuoka 812 8581, Japan
    Genes Cells 18:476-92. 2013
    ..Thus, peroxisomal protein import can be readily quantified by measuring the fluorescence intensity of whole cells...
  48. pmc Human PEX19: cDNA cloning by functional complementation, mutation analysis in a patient with Zellweger syndrome, and potential role in peroxisomal membrane assembly
    Y Matsuzono
    Department of Biology, Faculty of Science, Kyushu University, Fukuoka 812 8581, Japan
    Proc Natl Acad Sci U S A 96:2116-21. 1999
    ..Moreover, Pex19p is apparently involved at the initial stage in peroxisome membrane assembly, before the import of matrix protein...
  49. ncbi Molecular anatomy of the peroxin Pex12p: ring finger domain is essential for Pex12p function and interacts with the peroxisome-targeting signal type 1-receptor Pex5p and a ring peroxin, Pex10p
    K Okumoto
    Department of Biology, Kyushu University Graduate School of Science, Fukuoka, Japan
    J Biol Chem 275:25700-10. 2000
    ..Hence, the RING finger peroxins are most likely to be involved in Pex5p-mediated matrix protein import into peroxisomes...
  50. pmc The peroxin pex3p initiates membrane assembly in peroxisome biogenesis
    K Ghaedi
    Department of Biology, Graduate School of Science, Kyushu University, Fukuoka 812 8581, Japan
    Mol Biol Cell 11:2085-102. 2000
    ..Thus, Pex3p assembles membrane vesicles before the matrix proteins are translocated...
  51. pmc PEX3 is the causal gene responsible for peroxisome membrane assembly-defective Zellweger syndrome of complementation group G
    K Ghaedi
    Department of Biology, Faculty of Sciences, Kyushu University, Fukuoka, Japan and CREST, Japan Science and Technology Corporation, Tokyo, Japan
    Am J Hum Genet 67:976-81. 2000
    ..These results provide evidence that PEX3 is a novel, pathogenic gene responsible for CG-G PBDs...
  52. pmc Phenotype-genotype relationships in peroxisome biogenesis disorders of PEX1-defective complementation group 1 are defined by Pex1p-Pex6p interaction
    S Tamura
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Higashi ku, Fukuoka 812 8581, Japan
    Biochem J 357:417-26. 2001
    ..Failure in Pex1p-Pex6p interaction gives rise to more severe abnormalities, such as those manifested by patients with ZS...
  53. doi Nuclear transport of peroxisome-proliferator activated receptor α
    Fumiko Iwamoto
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka, Japan
    J Biochem 149:311-9. 2011
    ..Moreover, the PPARα NLS binds well-known nuclear transporters, importin α and importin β, in a manner independent of DNA-binding activity...
  54. ncbi Transmembrane topology of the peroxin, Pex2p, an essential component for the peroxisome assembly
    T Harano
    Department of Biology, Faculty of Science, Kyushu University, Fukuoka, 812 8581, Japan
    J Biochem 125:1168-74. 1999
    ..Therefore, Pex2p is anchored to the peroxisomal membrane by two membrane-spanning segments, with its N- and C-terminal regions exposed to the cytosol...
  55. pmc PEX12, the pathogenic gene of group III Zellweger syndrome: cDNA cloning by functional complementation on a CHO cell mutant, patient analysis, and characterization of PEX12p
    K Okumoto
    Department of Biology, Faculty of Science, Kyushu University, Fukuoka 812 8581, Japan
    Mol Cell Biol 18:4324-36. 1998
    ..Moreover, truncation and site mutation studies, including patient PEX12 analysis, demonstrated that the cytoplasmically oriented N- and C-terminal parts of Pex12p are essential for biological function...
  56. pmc Hsp70 regulates the interaction between the peroxisome targeting signal type 1 (PTS1)-receptor Pex5p and PTS1
    T Harano
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, 6 10 1 Hakozaki, Higashi ku, Fukuoka 812 8581, Japan
    Biochem J 357:157-65. 2001
    ....
  57. ncbi Biogenesis of nonspecific lipid transfer protein and sterol carrier protein x: studies using peroxisome assembly-defective pex cell mutants
    H Otera
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812-8581, Japan
    J Biol Chem 276:2858-64. 2001
    ..It is likely, therefore, that modulation of the C-terminal PTS1 by the presequence gives rise to cytoplasmic localization of pre-nsLTP...
  58. ncbi Isolation, characterization and mutation analysis of PEX13-defective Chinese hamster ovary cell mutants
    R Toyama
    Department of Biology, Kyushu University Graduate School of Science, Fukuoka 812 8581, Japan
    Hum Mol Genet 8:1673-81. 1999
    ..Therefore, ZP128 and ZP150 are CHO cell lines with a phenotype of impaired PEX13...
  59. ncbi The peroxin Pex6p gene is impaired in peroxisomal biogenesis disorders of complementation group 6
    N Matsumoto
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka, Japan
    J Hum Genet 46:273-7. 2001
    ..Expression of PEX6 restored peroxisome assembly in fibroblasts from a CG6 PBD patient. This patient was a compound heterozygote for PEX6 gene alleles. Accordingly, by merging CG6 with CG4, human PBDs are now classified into 12CGs...
  60. pmc Peroxisome targeting signal type 1 (PTS1) receptor is involved in import of both PTS1 and PTS2: studies with PEX5-defective CHO cell mutants
    H Otera
    Department of Biology, Kyushu University Faculty of Science, Fukuoka, Japan
    Mol Cell Biol 18:388-99. 1998
    ..Both mutations were in the TPR domains (TPR1 and TPR6), suggesting the functional consequence of these domains in protein translocation. The implications of these mutations are discussed...
  61. ncbi Topogenesis of peroxisomal membrane protein requires a short, positively charged intervening-loop sequence and flanking hydrophobic segments. study using human membrane protein PMP34
    M Honsho
    Department of Biology, Faculty of Sciences, Kyushu University Graduate School, Fukuoka 812 8581, Japan
    J Biol Chem 276:9375-82. 2001
    ..Therefore, it is evident that the intervening basic loop plus three transmembrane segments of PMP34 function as a peroxisomal targeting and topogenic signal...
  62. ncbi Clofibrate-inducible, 28-kDa peroxisomal integral membrane protein is encoded by PEX11
    I Abe
    Department of Biology, Faculty of Science, Kyushu University, Fukuoka, Japan
    FEBS Lett 431:468-72. 1998
    ..Pex11p exposes both N- and C-terminal parts to the cytosol. PEX11 was not responsible for ten complementation groups of human peroxisome deficiency disorders...
  63. pmc Human PEX1 cloned by functional complementation on a CHO cell mutant is responsible for peroxisome-deficient Zellweger syndrome of complementation group I
    S Tamura
    Department of Biology, Faculty of Science, Kyushu University, Fukuoka 812 81, Japan
    Proc Natl Acad Sci U S A 95:4350-5. 1998
    ..Both PBDE-04 PEX1 cDNAs were defective in peroxisome-restoring activity when expressed in the patient fibroblasts as well as in ZP107 cells. These results demonstrate that PEX1 is the causative gene for CG-I peroxisomal disorders...
  64. ncbi A cytoplasmic AAA family peroxin, Pex1p, interacts with Pex6p
    S Tamura
    Department of Biology, Faculty of Science, Kyushu University, Fukuoka, Japan
    Biochem Biophys Res Commun 245:883-6. 1998
    ..Conversely, 35S-Pex1p was obtained in immunoprecipitate from CHO-K1 expressing human Pex6p, using anti-Pex6p antibody. These results strongly suggest that Pex1p and Pex6p interact with each other...
  65. ncbi cDNA cloning and characterization of a constitutively expressed isoform of the human peroxin Pex11p
    I Abe
    Department of Biology, Faculty of Science, Kyushu University, Fukuoka, 812 8581, Japan
    Biochem Biophys Res Commun 252:529-33. 1998
    ..PEX11beta was not induced in rats by treatment of clofibrate, a peroxisome proliferator, in contrast to PEX11alpha...
  66. pmc Mutation in PEX16 is causal in the peroxisome-deficient Zellweger syndrome of complementation group D
    M Honsho
    Department of Biology, Faculty of Science, Kyushu University, Fukuoka, Japan
    Am J Hum Genet 63:1622-30. 1998
    ..PBDD-01-derived PEX16 cDNA was defective in peroxisome-restoring activity when expressed in the patient's fibroblasts. These results demonstrate that mutation in PEX16 is the genetic cause of CG-D PBDs...
  67. ncbi The peroxin Pex14p. cDNA cloning by functional complementation on a Chinese hamster ovary cell mutant, characterization, and functional analysis
    N Shimizu
    Department of Biology, Kyushu University Faculty of Science, Fukuoka 812 8581, Japan
    J Biol Chem 274:12593-604. 1999
    ..Neither of these two mutant forms of Pex14p restored peroxisome biogenesis in ZP110 and ZP161, thereby demonstrating PEX14 to be responsible for peroxisome deficiency in CG14...
  68. ncbi Alterations in the molecular species of plasmalogen phospholipids and glycolipids due to peroxisomal dysfunction in Chinese hamster ovary-mutant Z65 cells by FABMS method
    Makiko Saito
    Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    J Chromatogr B Analyt Technol Biomed Life Sci 852:367-73. 2007
    ..Enhanced synthesis of glycolipids in Z65 cells was associated with an abundance of lignoceric acid-containing ones, suggesting a role of glycolipids in the retention of longer saturated fatty acids...
  69. doi Characterization of the interaction between recombinant human peroxin Pex3p and Pex19p: identification of TRP-104 IN Pex3p as a critical residue for the interaction
    Yasuhiko Sato
    Department of Structural Biology, Graduate School of Pharmaceutical Sciences, Kyoto University, 46 29 Yoshida Shimoadachi cho, Sakyo ku, Kyoto, Japan
    J Biol Chem 283:6136-44. 2008
    ..These findings suggest that the indole ring of Trp-104 directly interacts with Pex19p to facilitate the specific peroxisomal translocation of the Pex19p-PMP complexes...
  70. ncbi Molecular cloning of Chinese hamster ceramide glucosyltransferase and its enhanced expression in peroxisome-defective mutant Z65 cells
    Makiko Saito
    Department of Pediatrics, Graduate School of Medicine, The University of Tokyo, 7 3 1 Hongo, Bunkyo ku, Tokyo 113 8655, Japan
    Arch Biochem Biophys 403:171-8. 2002
    ..With an immunohistochemical procedure, GM3 was found to be more strongly expressed in the cell membrane of Z65 cells than in CHO-K1 cells...
  71. ncbi A novel aberrant splicing mutation of the PEX16 gene in two patients with Zellweger syndrome
    Nobuyuki Shimozawa
    Department of Pediatrics, Gifu University School of Medicine, Gifu 500 8076, Japan
    Biochem Biophys Res Commun 292:109-12. 2002
    ..As a consequence, the cell's ability to membrane synthesis and protein import is disrupted, which implies that the changed C terminus of the Pex16p in these patients likely affects its function...
  72. ncbi Overexpression of human acyl-CoA thioesterase upregulates peroxisome biogenesis
    Mitsuru Ishizuka
    Department of Anatomy and Developmental Biology, Graduate School of Medicine, Chiba University, Chiba 260 8670, Japan
    Exp Cell Res 297:127-41. 2004
    ..Thus, ACTEIII/PTE-1 may be involved in the metabolic regulation of peroxisome proliferation...