Although mitochondrial alterations and mistargeting of peroxisomal proteins to mitochondria have been observed in peroxisome biogenesis disorders (Nuebel et al., 2021; Peeters et al., 2015), it remains to be established whether PEX11β mistargeting contributes to the pathophysiology of PEX19-deficiency and related disorders. The gene discussed is PEX19; the disease is Peroxisome biogenesis disorder-Zellweger syndrome spectrum.