Individuals with peroxisome biogenesis disorders (PBDs) suffer from various symptoms, such as deafness, hepatic disease and mental disability. The range of symptoms indicates the importance of peroxisomes for normal physiology, but exactly how peroxisome defects cause these disorders is unclear. Mast et al. now show that Drosophila is a relevant model for addressing this issue by characterising flies carrying mutant Pex1 (the fly homologue of the gene most commonly mutated in humans with PBDs). Pex1 mutant flies have characteristics similar to patients with PBDs, and genetic profiling indicates that peroxisomal function is important for neuronal development, innate immunity, lipid and protein metabolism, and gamete formation. Page 659
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