PEX5L

Summary

Gene Symbol: PEX5L
Description: peroxisomal biogenesis factor 5 like
Alias: PEX5R, PEX5RP, PXR2, PXR2B, TRIP8b, PEX5-related protein, HCN channel auxiliary subunit, PEX2-related protein, PEX5-like protein, Pex5p-related protein, peroxin-5-related protein, peroxisome biogenesis factor 5-like, tetratricopeptide repeat-containing Rab8b-interacting protein
Species: human

Top Publications

  1. pmc Identification of PEX5p-related novel peroxisome-targeting signal 1 (PTS1)-binding proteins in mammals
    L Amery
    Katholieke Universiteit Leuven, Campus Gasthuisberg O N, Departement Moleculaire Celbiologie, Afdeling Farmacologie, Herestraat 49, B 3000 Leuven, Belgium
    Biochem J 357:635-46. 2001
  2. doi A cell-free organelle-based in vitro system for studying the peroxisomal protein import machinery
    Tony A Rodrigues
    Instituto de Investigação e Inovação em Saúde i3S, Universidade do Porto, Porto, Portugal
    Nat Protoc 11:2454-2469. 2016
  3. pmc PXR variants: the impact on drug metabolism and therapeutic responses
    C Trent Brewer
    Department of Chemical Biology and Therapeutics, St Jude Children s Research Hospital, Memphis, TN 38105, USA Integrated Biomedical Sciences Program, University of Tennessee Health Science Center, Memphis, TN 38163, USA
    Acta Pharm Sin B 6:441-449. 2016
  4. doi A novel type of rhizomelic chondrodysplasia punctata, RCDP5, is caused by loss of the PEX5 long isoform
    Tuva Barøy
    Department of Medical Genetics and
    Hum Mol Genet 24:5845-54. 2015
  5. pmc Mechanistic insights into PTS2-mediated peroxisomal protein import: the co-receptor PEX5L drastically increases the interaction strength between the cargo protein and the receptor PEX7
    Markus Kunze
    From the Center for Brain Research, Medical University of Vienna, Spitalgasse 4, 1090 Vienna, Austria
    J Biol Chem 290:4928-40. 2015
  6. pmc Structural basis for the mutual antagonism of cAMP and TRIP8b in regulating HCN channel function
    Andrea Saponaro
    Department of Biosciences, University of Milan, 20133 Milan, Italy
    Proc Natl Acad Sci U S A 111:14577-82. 2014
  7. doi Nedd4-2 regulates surface expression and may affect N-glycosylation of hyperpolarization-activated cyclic nucleotide-gated (HCN)-1 channels
    Wiebke Wilkars
    1Institute of Neuroanatomy, University of Hamburg Medical Center, Martinistr 52, 20246 Hamburg, Germany
    FASEB J 28:2177-90. 2014
  8. pmc Structure and stoichiometry of an accessory subunit TRIP8b interaction with hyperpolarization-activated cyclic nucleotide-gated channels
    John R Bankston
    Department of Physiology and Biophysics, University of Washington School of Medicine, Seattle, WA 98195, USA
    Proc Natl Acad Sci U S A 109:7899-904. 2012
  9. pmc The molecular biology of vestibular schwannomas and its association with hearing loss: a review
    Erika Celis-Aguilar
    Instituto Nacional de Neurologia y Neurocirugia Manuel Velasco Suarez, 14269 Ciudad de México, DF, Mexico
    Genet Res Int 2012:856157. 2012
  10. pmc TRIP8b-independent trafficking and plasticity of adult cortical presynaptic HCN1 channels
    Zhuo Huang
    Department of Pharmacology, UCL School of Pharmacy, University College London, London, WC1N 1AX, United Kingdom
    J Neurosci 32:14835-48. 2012

Research Grants

  1. Neural Calcium Channels-Regulation and Function
    Diane Lipscombe; Fiscal Year: 2009

Scientific Experts

  • Luis Lassaletta
  • Markus Kunze
  • Roland A Bender
  • Marc Fransen
  • B Santoro
  • Diane Lipscombe
  • G Dodt
  • Dane M Chetkovich
  • Alan S Lewis
  • Merel S Ebberink
  • Tony A Rodrigues
  • C Trent Brewer
  • Tuva Barøy
  • Wiebke Wilkars
  • Andrea Saponaro
  • Janet Koster
  • Erika Celis-Aguilar
  • Ronald J A Wanders
  • John R Bankston
  • Zhuo Huang
  • Hans R Waterham
  • Ye Han
  • Xueling Sim
  • Masanori Honsho
  • Gerd Zolles
  • Yukio Fujiki
  • Non Miyata
  • Xiaojian Wang
  • Cláudia P Grou
  • Ana F Dias
  • Tânia Francisco
  • Jorge E Azevedo
  • Taosheng Chen
  • Ana G Pedrosa
  • Nick Wood
  • Timothy Hughes
  • Kristine Stadskleiv
  • Asbjørn Holmgren
  • Eirik Frengen
  • John Walter
  • Berit Woldseth
  • Doriana Misceo
  • Sacha Ferdinandusse
  • Petter Strømme
  • Else Merckoll
  • Jostein Westvik
  • Bjørn Tvedt
  • Lei Hu
  • Manolis Matzapetakis
  • Francesca Cantini
  • Robert Bähring
  • Anna Moroni
  • Mieri Han
  • Gerhard Thiel
  • Christian Hammann
  • Evita Mohr
  • Sofia R Pauleta
  • Lucia Banci
  • Chiara Donadoni
  • Jessica Wollberg
  • Javier S Castresana
  • Jose Martínez-Hernández
  • Javier Gavilan
  • L Amery
  • Mala M Shah
  • Frank DiMaio
  • Manuel Nistal
  • Miguel Torres-Martin
  • F Yuri Rodrigues
  • William N Zagotta
  • Rafael Lujan
  • Juan A Rey
  • Stacey S Camp
  • Wan Ting Tay
  • Wytse J Wadman
  • Kee Seng Chia
  • Chen Suo
  • Mark Seielstad
  • Daniel Peng Keat Ng
  • Michael Boehnke
  • Yasuko Hashiguchi
  • Jianjun Liu
  • Rick Twee Hee Ong
  • Johnie J Gallagher
  • Tien Yin Wong
  • Tallie Z Baram
  • Yik Ying Teo
  • Kamran Ghaedi
  • E Shyong Tai
  • Yoav Noam

Detail Information

Publications23

  1. pmc Identification of PEX5p-related novel peroxisome-targeting signal 1 (PTS1)-binding proteins in mammals
    L Amery
    Katholieke Universiteit Leuven, Campus Gasthuisberg O N, Departement Moleculaire Celbiologie, Afdeling Farmacologie, Herestraat 49, B 3000 Leuven, Belgium
    Biochem J 357:635-46. 2001
    ..in an in vitro peroxisome-targeting signal 1 (PTS1)-binding assay, we refer to them as 'Pex5p-related proteins' (Pex5Rp)...
  2. doi A cell-free organelle-based in vitro system for studying the peroxisomal protein import machinery
    Tony A Rodrigues
    Instituto de Investigação e Inovação em Saúde i3S, Universidade do Porto, Porto, Portugal
    Nat Protoc 11:2454-2469. 2016
    ..In addition, a receptor (PEX5L/S or PEX5L.PEX7) can be used to report the dynamics of shuttling proteins that mediate the import process...
  3. pmc PXR variants: the impact on drug metabolism and therapeutic responses
    C Trent Brewer
    Department of Chemical Biology and Therapeutics, St Jude Children s Research Hospital, Memphis, TN 38105, USA Integrated Biomedical Sciences Program, University of Tennessee Health Science Center, Memphis, TN 38163, USA
    Acta Pharm Sin B 6:441-449. 2016
    ..variants 3 (PXR3) and 4 (PXR4) do not induce target gene expression, whereas transcript variants 1 (PXR1) and 2 (PXR2) respond to agonist by activating target gene expression...
  4. doi A novel type of rhizomelic chondrodysplasia punctata, RCDP5, is caused by loss of the PEX5 long isoform
    Tuva Barøy
    Department of Medical Genetics and
    Hum Mol Genet 24:5845-54. 2015
    ..722dupA (p.Val242Glyfs(∗)33) in PEX5 (GenBank: NM_001131023.1). PEX5 encodes two isoforms, PEX5L and PEX5S, and we show that the c...
  5. pmc Mechanistic insights into PTS2-mediated peroxisomal protein import: the co-receptor PEX5L drastically increases the interaction strength between the cargo protein and the receptor PEX7
    Markus Kunze
    From the Center for Brain Research, Medical University of Vienna, Spitalgasse 4, 1090 Vienna, Austria
    J Biol Chem 290:4928-40. 2015
    ..transport to peroxisomes by a concerted action with a co-receptor protein, which in mammals is the PTS1 receptor PEX5L. Using a modified version of the mammalian two-hybrid assay, we demonstrate that the interaction strength between ..
  6. pmc Structural basis for the mutual antagonism of cAMP and TRIP8b in regulating HCN channel function
    Andrea Saponaro
    Department of Biosciences, University of Milan, 20133 Milan, Italy
    Proc Natl Acad Sci U S A 111:14577-82. 2014
    ..Neuronal HCN channels are also regulated by tetratricopeptide repeat-containing Rab8b interacting protein (TRIP8b), an auxiliary subunit that antagonizes the effects of cAMP by interacting with the channel CNBD...
  7. doi Nedd4-2 regulates surface expression and may affect N-glycosylation of hyperpolarization-activated cyclic nucleotide-gated (HCN)-1 channels
    Wiebke Wilkars
    1Institute of Neuroanatomy, University of Hamburg Medical Center, Martinistr 52, 20246 Hamburg, Germany
    FASEB J 28:2177-90. 2014
    ..in HEK293 cells, thereby opposing effects of tetratricopeptide repeat-containing Rab8b interacting protein (TRIP8b)-(1a-4), an auxiliary subunit that promotes HCN1 surface expression...
  8. pmc Structure and stoichiometry of an accessory subunit TRIP8b interaction with hyperpolarization-activated cyclic nucleotide-gated channels
    John R Bankston
    Department of Physiology and Biophysics, University of Washington School of Medicine, Seattle, WA 98195, USA
    Proc Natl Acad Sci U S A 109:7899-904. 2012
    ..b>TRIP8b (tetratricopetide repeat-containing Rab8b-interacting protein) is a recently discovered accessory subunit of ..
  9. pmc The molecular biology of vestibular schwannomas and its association with hearing loss: a review
    Erika Celis-Aguilar
    Instituto Nacional de Neurologia y Neurocirugia Manuel Velasco Suarez, 14269 Ciudad de México, DF, Mexico
    Genet Res Int 2012:856157. 2012
    ..cyclin D1, expression of B7-H1, increased expression of the platelet-derived growth factor A, underexpression of PEX5L, RAD54B, and PSMAL, and overexpression of CEA...
  10. pmc TRIP8b-independent trafficking and plasticity of adult cortical presynaptic HCN1 channels
    Zhuo Huang
    Department of Pharmacology, UCL School of Pharmacy, University College London, London, WC1N 1AX, United Kingdom
    J Neurosci 32:14835-48. 2012
    ..HCN channel trafficking to dendrites is regulated by the protein, TRIP8b. Additionally, altered TRIP8b expression may be one mechanism underlying seizure-induced dendritic HCN channel ..
  11. doi Comparison of the PTS1- and Rab8b-binding properties of Pex5p and Pex5Rp/TRIP8b
    Marc Fransen
    Katholieke Universiteit Leuven, Campus Gasthuisberg O and N 1, Departement Moleculaire Celbiologie, LIPIT, Leuven, Belgium
    Biochim Biophys Acta 1783:864-73. 2008
    ..Rab8b, and its human orthologue as a protein related to the peroxisomal targeting signal 1 (PTS1) receptor Pex5p (Pex5Rp)...
  12. doi Genotype-phenotype correlation in PEX5-deficient peroxisome biogenesis defective cell lines
    Merel S Ebberink
    Laboratory Genetic Metabolic Diseases, Department of Clinical Chemistry, Academic Medical Centre, University of Amsterdam, Amsterdam, The Netherlands
    Hum Mutat 30:93-8. 2009
    ..Alternative splicing of PEX5 results in two protein variants, PEX5S and PEX5L. PEX5S is exclusively involved in PTS1 protein import, whereas PEX5L mediates the import of both PTS1 and PTS2 ..
  13. doi Tripping the HCN breaker
    Diane Lipscombe
    Department of Neuroscience, Brown University, Providence, RI 02912, USA
    Neuron 62:747-50. 2009
    ..In this issue of Neuron two manuscripts (Santoro et al. and Zolles et al.) report that HCN channel gating and expression are controlled by Trip8b (Pex5R) but with a bidirectional spin.
  14. doi Association with the auxiliary subunit PEX5R/Trip8b controls responsiveness of HCN channels to cAMP and adrenergic stimulation
    Gerd Zolles
    Institute of Physiology, University of Freiburg, Engesserstrasse 4, 79108 Freiburg, Germany
    Neuron 62:814-25. 2009
    ..Using affinity purification and high-resolution mass spectrometry, we identify the PEX5R/Trip8b protein as the beta subunit of HCN channels in the mammalian brain...
  15. 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
    ..1 nt upstream of exon 7 encoding Pex5pL specific 37-amino acid insertion, thereby generating 21-nt deleted mRNA of PEX5L in ZPEG241...
  16. pmc TRIP8b regulates HCN1 channel trafficking and gating through two distinct C-terminal interaction sites
    Bina Santoro
    Department of Neuroscience and Pharmacology, Howard Hughes Medical Institute, Columbia University, New York, New York 10032, USA
    J Neurosci 31:4074-86. 2011
    ..cyclic nucleotide-regulated (HCN) channels in the brain associate with their auxiliary subunit TRIP8b (also known as PEX5R), a cytoplasmic protein expressed as a family of alternatively spliced isoforms...
  17. pmc Transferability of type 2 diabetes implicated loci in multi-ethnic cohorts from Southeast Asia
    Xueling Sim
    Centre for Molecular Epidemiology, National University of Singapore, Singapore, Singapore
    PLoS Genet 7:e1001363. 2011
    ..These findings highlight the value of surveying across diverse racial/ethnic groups towards the fine-mapping efforts for the casual variants and also of the search for variants, which may be population-specific...
  18. pmc Trafficking and gating of hyperpolarization-activated cyclic nucleotide-gated channels are regulated by interaction with tetratricopeptide repeat-containing Rab8b-interacting protein (TRIP8b) and cyclic AMP at distinct sites
    Ye Han
    Davee Department of Neurology and Clinical Neurosciences, Northwestern University Feinberg School of Medicine, Chicago, Illinois 60611, USA
    J Biol Chem 286:20823-34. 2011
    ..Tetratricopeptide repeat-containing Rab8b-interacting protein (TRIP8b) is an auxiliary subunit for neuronal hyperpolarization-activated cyclic nucleotide-gated (HCN) channels...
  19. 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
    ..The longer isoform of Pex5, Pex5L, also transports Pex7-PTS2 protein complexes. After unloading the cargoes, Pex5 returns to the cytosol...
  20. ncbi Regulation of HCN channel surface expression by a novel C-terminal protein-protein interaction
    Bina Santoro
    Center for Neurobiology and Behavior, Columbia University, New York, New York 10032, USA
    J Neurosci 24:10750-62. 2004
    ..We identified a brain-specific protein, which has been previously termed TRIP8b (for TPR-containing Rab8b interacting protein) and PEX5Rp (for Pex5p-related protein), that specifically interacts ..
  21. ncbi A novel human phosphatidylethanolamine-binding protein resists tumor necrosis factor alpha-induced apoptosis by inhibiting mitogen-activated protein kinase pathway activation and phosphatidylethanolamine externalization
    Xiaojian Wang
    Institute of Immunology, Zhejiang University, Hangzhou 310031, People s Republic of China
    J Biol Chem 279:45855-64. 2004
    ....
  22. ncbi Domain mapping of human PEX5 reveals functional and structural similarities to Saccharomyces cerevisiae Pex18p and Pex21p
    G Dodt
    Institut fur Physiologische Chemie, Systembiochemie Ruhr Universität, 44801 Bochum, Germany
    J Biol Chem 276:41769-81. 2001
    ..Human cells generate two isoforms of PEX5 through alternative splicing, PEX5S and PEX5L, and PEX5L contains an additional insert 37 amino acids long...
  23. ncbi An isoform of pex5p, the human PTS1 receptor, is required for the import of PTS2 proteins into peroxisomes
    N Braverman
    Department of Pediatrics, Ruhr Universitat, Bochum, Germany q
    Hum Mol Genet 7:1195-205. 1998
    ..PEX5 expression and found that it undergoes alternative splicing, producing two transcripts, one containing (PEX5L) and one lacking (PEX5S) a 111 bp internal exon...

Research Grants1

  1. Neural Calcium Channels-Regulation and Function
    Diane Lipscombe; Fiscal Year: 2009
    ..2. This novel interaction is likely to mediate coupling of the CaV2.2 channel to the synaptic release machinery (aim 3). ..