Cargo of PEX5S,L translocates from the cytosol to the peroxisomal matrix

Stable Identifier
Reaction [uncertain]
Homo sapiens
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After binding the Docking and Translocation Module comprising PEX14, PEX13, PEX2, PEX10 and PEX12 , PEX5S or PEX5L bound to a cargo protein becomes localized to the membrane (Dodt et al. 1995, Wiemer et al. 1995, Alencastre et al. 2009, Francisco et al. 2013, Dias et al. 2017). In a reaction that is not yet fully characterized, the cargo protein is released into the peroxisomal matrix while PEX5S or PEX5L remains in the membrane (Dodt et al. 1995, Wiemer et al. 1995, Alencastre et al. 2009, Franscisco et al. 2013). One model for the reaction hypothesizes that PEX13:PEX14 (associated with PEX2:PEX10:PEX12) forms a barrel in the peroxisomal membrane while PEX5S or PEX5L acts as a plunger to guide the cargo through the barrel (Dias et al. 2017, Francisco et al. 2017). Notably, the reaction does not require a source of energy such as ATP (Oliveira et al. 2003). 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
26220973 A novel type of rhizomelic chondrodysplasia punctata, RCDP5, is caused by loss of the PEX5 long isoform

Barøy, T, Koster, J, Strømme, P, Ebberink, MS, Misceo, D, Ferdinandusse, S, Holmgren, A, Hughes, T, Merckoll, E, Westvik, J, Woldseth, B, Walter, J, Wood, N, Tvedt, B, Stadskleiv, K, Wanders, RJ, Waterham, HR, Frengen, E

Hum. Mol. Genet. 2015
7719337 Mutations in the PTS1 receptor gene, PXR1, define complementation group 2 of the peroxisome biogenesis disorders

Dodt, G, Braverman, N, Wong, C, Moser, A, Moser, HW, Watkins, P, Valle, D, Gould, SJ

Nat. Genet. 1995
7790377 Human peroxisomal targeting signal-1 receptor restores peroxisomal protein import in cells from patients with fatal peroxisomal disorders

Wiemer, EA, Nuttley, WM, Bertolaet, BL, Li, X, Francke, U, Wheelock, MJ, Anné, UK, Johnson, KR, Subramani, S

J. Cell Biol. 1995
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
12885776 The energetics of Pex5p-mediated peroxisomal protein import

Oliveira, ME, Gouveia, AM, Pinto, RA, Sá-Miranda, C, Azevedo, JE

J. Biol. Chem. 2003
28787099 Protein transport into peroxisomes: Knowns and unknowns

Francisco, T, Rodrigues, TA, Dias, AF, Barros-Barbosa, A, Bicho, D, Azevedo, JE

Bioessays 2017
19632994 Mapping the cargo protein membrane translocation step into the PEX5 cycling pathway

Alencastre, IS, Rodrigues, TA, Grou, CP, Fransen, M, Sá-Miranda, C, Azevedo, JE

J. Biol. Chem. 2009
23963456 A cargo-centered perspective on the PEX5 receptor-mediated peroxisomal protein import pathway

Francisco, T, Rodrigues, TA, Freitas, MO, Grou, CP, Carvalho, AF, Sá-Miranda, C, Pinto, MP, Azevedo, JE

J. Biol. Chem. 2013
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
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