Mouse Models to Study Peroxisomal Functions and Disorders: Overview, Caveats, and Recommendations
- PMID: 36952207
- DOI: 10.1007/978-1-0716-3048-8_34
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.
Keywords: Bile acids; Knockout; Mouse; Peroxisomes; Plasmalogens; VLCFA.
© 2023. The Author(s), under exclusive license to Springer Science+Business Media, LLC, part of Springer Nature.
References
-
- Waterham HR, Ferdinandusse S, Wanders RJ (2016) Human disorders of peroxisome metabolism and biogenesis. Biochim Biophys Acta 1863(5):922–933. https://doi.org/10.1016/j.bbamcr.2015.11.015 - DOI - PubMed
-
- Baes M, Van Veldhoven PP (2012) Mouse models for peroxisome biogenesis defects and beta-oxidation enzyme deficiencies. Biochim Biophys Acta 1822(9):1489–1500. https://doi.org/10.1016/j.bbadis.2012.03.003 - DOI - PubMed
-
- Yamashita S, Abe K, Tatemichi Y, Fujiki Y (2014) The membrane peroxin PEX3 induces peroxisome-ubiquitination-linked pexophagy. Autophagy 10(9):1549–1564. https://doi.org/10.4161/auto.29329 - DOI - PubMed - PMC
-
- Reuter M, Kooshapur H, Suda JG, Gaussmann S, Neuhaus A, Bruhl L, Bharti P, Jung M, Schliebs W, Sattler M, Erdmann R (2021) Competitive microtubule binding of PEX14 coordinates peroxisomal protein import and motility. J Mol Biol 433(5):166765. https://doi.org/10.1016/j.jmb.2020.166765 - DOI - PubMed
-
- Baes M, Gressens P, Baumgart E, Carmeliet P, Casteels M, Fransen M, Evrard P, Fahimi D, Declercq PE, Collen D, van Veldhoven PP, Mannaerts GP (1997) A mouse model for Zellweger syndrome. Nat Genet 17(1):49–57. https://doi.org/10.1038/ng0997-49 - DOI - PubMed
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