Mouse Models to Study Peroxisomal Functions and Disorders: Overview, Caveats, and Recommendations

Abstract

During the last three decades many mouse lines were created or identified that are deficient in one or more peroxisomal functions. Different methodologies were applied to obtain global, hypomorph, cell type selective, inducible and knockin mice. Whereas some models closely mimic pathologies in patients, others strongly deviate or no human counterpart has been reported. Often, mice, apparently endowed with a stronger transcriptional adaptation, have to be challenged with dietary additions or restrictions in order to trigger phenotypic changes. Depending on the inactivated peroxisomal protein, several approaches can be taken to validate the loss-of-function. Here, an overview is given of the available mouse models and their most important characteristics.

The peroxisome: an update on mysteries 3.0

“…Mouse models have been successfully used to study peroxisomal functions and disorders (summarised in Kocherlakota et al 2023b ). Another popular vertebrate model organism for developmental and neurobiology is the zebrafish ( Danio rerio ), due to its high degree of genome conservation with mammals ( D. rerio ).…”