Journal Article

DKFZ-2025-01603

http://join2-wiki.gsi.de/foswiki/pub/Main/Artwork/join2_logo100x88.png PEX39 facilitates the peroxisomal import of PTS2-containing proteins.

Chen, W. W. ; Rodrigues, T. A. ; Wendscheck, D. ; Pedrosa, A. G. ; Yang, C. ; Francisco, T. ; Möcklinghoff, T.DKFZ* ; Zografakis, A. ; Nunes-Silva, B. ; Avraham, R. E. ; Silva, A. R. ; Ferreira, M. J. ; Das, H. ; Koster, J. ; Neuwirth, S. ; Bender, J. ; Oeljeklaus, S. ; Sondhi, V. ; Gatsogiannis, C. ; Schuldiner, M. ; Zalckvar, E. ; Hofmann, K. ; Waterham, H. R. ; DeBerardinis, R. J. ; Azevedo, J. E. ; Warscheid, B.

2025
Nature America New York, NY

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Please use a persistent id in citations: doi:10.1038/s41556-025-01711-z

Abstract: Peroxisomes are metabolic organelles essential for human health. Defects in peroxisomal biogenesis proteins (also known as peroxins (PEXs)) cause devastating disease. PEX7 binds proteins containing a type 2 peroxisomal targeting signal (PTS2) to enable their import from the cytosol into peroxisomes, although many aspects of this process remain enigmatic. Utilizing in vitro assays, yeast and human cells, we show that PEX39, a previously uncharacterized protein, is a cytosolic peroxin that facilitates the import of PTS2-containing proteins by binding PEX7 and stabilizing its interaction with cargo proteins containing a PTS2. PEX39 and PEX13, a peroxisomal membrane translocon protein, both possess an (R/K)PWE motif necessary for PEX7 binding. Handover of PEX7 from PEX39 to PEX13 via these motifs provides a new paradigm for peroxisomal protein import and biogenesis. Collectively, this work reveals how PEX39 and (R/K)PWE motifs facilitate the import of PTS2-containing proteins and advances our understanding of peroxisomal disease.

Note: 2025 Aug;27(8):1256-1271

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Contributing Institute(s):

1. B200 Systembiologie der Signaltransduktion (B200)

Research Program(s):

1. 312 - Funktionelle und strukturelle Genomforschung (POF4-312) (POF4-312)

Appears in the scientific report 2025

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Database coverage:
; BIOSIS Previews ; Biological Abstracts ; Clarivate Analytics Master Journal List ; Current Contents - Life Sciences ; DEAL Nature ; Ebsco Academic Search ; Essential Science Indicators ; IF >= 20 ; JCR ; Nationallizenz ; SCOPUS ; Science Citation Index Expanded ; Web of Science Core Collection

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