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Article

Unbalanced lipolysis results in lipotoxicity and mitochondrial damage in peroxisome-deficient Pex19 mutants.

Molecular biology of the cell - 15 Feb 2018

Bülow Margret H, Wingen Christian, Senyilmaz Deniz, Gosejacob Dominic, Sociale Mariangela, Bauer Reinhard, Schulze Heike, Sandhoff Konrad, Teleman Aurelio A, Hoch Michael, Sellin Julia

Abstract excerpt

Inherited peroxisomal biogenesis disorders (PBDs) are characterized by the absence of functional peroxisomes. They are caused by mutations of peroxisomal biogenesis factors encoded by Pex genes, and result in childhood lethality. Owing to the many metabolic functions fulfilled by peroxisomes, PBD pathology is complex and incompletely understood. Besides accumulation of peroxisomal educts (like very-long-chain...

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PMID: 29282281
DOI: 10.1091/mbc.E17-08-0535
PMCID: PMC6014165

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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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