PEX10 is an E3 ubiquitin-protein ligase component of a peroxisomal retrotranslocation channel essential for peroxisome organization 1. The channel comprises three RING finger proteins (PEX2, PEX10, PEX12) that co-assemble transmembrane segments into an open pore, with PEX10's ring finger domain positioned above the channel 1. PEX10 mediates recycling of the PEX5 receptor from peroxisomes to cytosol by monoubiquitylating receptor N-termini during extraction through the pore 21. When recycling is compromised, PEX10 catalyzes polyubiquitylation of PEX5, leading to its proteasomal degradation and maintaining peroxisomal import factor homeostasis 1. Mutations in PEX10 cause peroxisome biogenesis disorders 6A and 6B, which present with cerebellar atrophy, ataxia, mental retardation, and neurological dysfunction 3. Hypomorphic PEX10 variants can cause milder phenotypes with translation reinitiation mechanisms 4. Beyond canonical peroxisomal function, PEX10 expression is regulated by androgen receptor signaling in prostate cancer, where enzalutamide-mediated PEX10 inhibition sensitizes cells to reactive oxygen species activators, suggesting therapeutic potential 56. PEX10 polymorphisms are associated with nonobstructive azoospermia susceptibility 7.