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. 2016 Nov 7;1(2):111-144.
doi: 10.3233/TRD-160003.

Peroxisome biogenesis disorders

Catherine Argyriou  1 , Maria Daniela D'Agostino  2 , Nancy Braverman  3
Affiliations

Peroxisome biogenesis disorders

Catherine Argyriou et al. Transl Sci Rare Dis. .
No abstract available

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Figures

Fig.1
Fig.1
Peroxisome matrix protein import. (1) PTS receptor binding. PEX5 and PEX7 cytosolic receptors bind their cognate ligands (PTS1 and PTS2 enzymes, respectively) in the cytosol. PEX5 has two isoforms that differ by alternative splicing. The longer isoform, PEX5L, binds both PTS1 enzymes and PEX7 and delivers them to the peroxisome membrane. (2) Docking. The receptor-ligand complex docks at the peroxisome membrane by binding PEX13 and PEX14. (3) Matrix enzyme translocation. PEX5, together with PEX14, forms a dynamic membrane pore through which the ligands are transported into the peroxisome matrix. (4) Receptor recycling. PEX2, PEX10, and PEX12 mono-ubiquitinate PEX5, allowing its removal from the membrane. The PEX1-PEX6 AAA-ATPase heterohexamer (anchored to the peroxisome membrane by PEX26) uses the energy from ATP hydrolysis to remove PEX5-Ub from the peroxisome membrane for another round of import. PEX7 is recycled to the cytosol after PEX5 in an ATP independent manner. Note that defects in PEX7 prevent import of PTS2 enzymes, but do not disrupt the PEX5/PTS1 import pathway.
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