Cargo of PEX5L:PEX7 translocates from the cytosol to the peroxisomal matrix

Stable Identifier
R-HSA-9033514
Type
Reaction [uncertain]
Species
Homo sapiens
Compartment
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The cargo protein bound to PEX7 is released from PEX7 into the peroxisomal matrix in a reaction that does not require ATP (Purdue et al. 1997, Dodt et al. 2001, Rodrigues et al. 2014, Rodrigues et al. 2015). PEX7 may also be released into the matrix (inferred from yeast in Nair et al. 2004), however later research indicates that PEX7 remains with PEX5L in the peroxisomal membrane (Rodrigues et al. 2015) apparently in a proteinaceous cavity (Dias et al. 2017). Mutations in PEX5 cause defects in import of PTS1-containing proteins or PTS2-containing proteins or both (Eberrink et al. 2009, Barøy et al. 2015).

Literature References
PubMed ID Title Journal Year
9090383 Rhizomelic chondrodysplasia punctata is caused by deficiency of human PEX7, a homologue of the yeast PTS2 receptor

Purdue, PE, Zhang, JW, Skoneczny, M, Lazarow, PB

Nat. Genet. 1997
15545321 Pex7p translocates in and out of peroxisomes in Saccharomyces cerevisiae

Nair, DM, Purdue, PE, Lazarow, PB

J. Cell Biol. 2004
18712838 Genotype-phenotype correlation in PEX5-deficient peroxisome biogenesis defective cell lines

Ebberink, MS, Mooyer, PA, Koster, J, Dekker, CJ, Eyskens, FJ, Dionisi-Vici, C, Clayton, PT, Barth, PG, Wanders, RJ, Waterham, HR

Hum. Mutat. 2009
24865970 A PEX7-centered perspective on the peroxisomal targeting signal type 2-mediated protein import pathway

Rodrigues, TA, Alencastre, IS, Francisco, T, Brites, P, Fransen, M, Grou, CP, Azevedo, JE

Mol. Cell. Biol. 2014
26138649 Revisiting the intraperoxisomal pathway of mammalian PEX7

Rodrigues, TA, Grou, CP, Azevedo, JE

Sci Rep 2015
28765278 The peroxisomal matrix protein translocon is a large cavity-forming protein assembly into which PEX5 protein enters to release its cargo

Dias, AF, Rodrigues, TA, Pedrosa, AG, Barros-Barbosa, A, Francisco, T, Azevedo, JE

J. Biol. Chem. 2017
11546814 Domain mapping of human PEX5 reveals functional and structural similarities to Saccharomyces cerevisiae Pex18p and Pex21p

Dodt, G, Warren, D, Becker, E, Rehling, P, Gould, SJ

J. Biol. Chem. 2001
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