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Article

Longitudinal study of Pex1-G844D NMRI mouse model: A robust pre-clinical model for mild Zellweger spectrum disorder.

Biochimica et biophysica acta. Molecular basis of disease - 1 Nov 2020

Demaret Tanguy, Roumain Martin, Ambroise Jérôme, Evraerts Jonathan, Ravau Joachim, Bouzin Caroline, Bearzatto Bertrand, Gala Jean-Luc, Stepman Hedwig, Marie Sandrine, Vincent Marie-Françoise, Muccioli Giulio G, Najimi Mustapha, Sokal Etienne M

Abstract excerpt

Zellweger spectrum disorders (ZSD) are inborn errors of metabolism caused by mutations in PEX genes that lead to peroxisomal biogenesis disorder (PBD). No validated treatment is able to modify the dismal progression of the disease. ZSD mouse models used to develop therapeutic approaches are limited by poor survival and breeding restrictions. To overcome these limitations, we backcrossed the hypomorphic Pex1...

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PMID: 32693164
DOI: 10.1016/j.bbadis.2020.165900

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PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 7 Sept 2026. Manifest publication-graph-cohort-2026-09-07.1.

Source receipt: pubmed, retrieved 7 Sept 2026.

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