Absence of peroxisomes in mouse hepatocytes causes mitochondrial and ER abnormalities†
Open accessAbstract
Peroxisome deficiency in men causes severe pathology in several organs, particularly in the brain and liver, but it is still unknown how metabolic abnormalities trigger these defects. In the present study, a mouse model with hepatocyte-selective elimination of peroxisomes was generated by inbreeding Pex5-loxP and albumin-Cre mice to investigate the consequences of peroxisome deletion on the functioning of hepatocytes. Besides the absence of catalase-positive peroxisomes, multiple ultrastructural alterations were noticed, including hepatocyte hypertrophy and hyperplasia, smooth endoplasmic reticulum proliferation, and accumulation of lipid droplets and lysosomes. Most prominent was the abnormal structure of the inner mitochondrial membrane, which bore some similarities with changes observed in Zellweger patients. This was accompanied by severely reduced activities of complex I, III, and V and a collapse of the mitochondrial inner membrane potential. Surprisingly, these abnormalities provoked no significant disturbances of adenosine triphosphate (ATP) levels and redox state of the liver. However, a compensatory increase of glycolysis as an alternative source of ATP and mitochondrial proliferation were observed. No evidence of oxidative damage to proteins or lipids nor elevation of oxidative stress defence mechanisms were found. Altered expression of peroxisome proliferator-activated receptor alpha (PPAR-alpha) regulated genes indicated that PPAR-alpha is activated in the peroxisome-deficient cells. In conclusion, the absence of peroxisomes from mouse hepatocytes has an impact on several other subcellular compartments and metabolic pathways but is not detrimental to the function of the liver parenchyma. Supplementary material for this article can be found on the HEPATOLOGY website (http://interscience.wiley.com/jpages/0270-9139/suppmat/index.html).
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- Authors
- Dirkx, R., Vanhorebeek, I., Martens, K., Schad, A., Grabenbauer, M., Fahimi, D., Declercq, P., Van Veldhoven, P. P., & Baes, M.
- Affiliations
- KU Leuven (BE)
- Heidelberg University (DE)
- Corresponding author
- Baes, Myriam
- Venue
- Hepatology
- Published
- 2005-04
- Volume
- 41
- Issue
- 4
- Pages
- 868-878
- ISSN
- 0270-9139 (ISSN-L)
- OpenAlex
- W2040365013
- Cited by
- 174
- References
- 49
- Citations by year
- 2026: 2 · 2025: 6 · 2024: 3 · 2023: 11 · 2022: 8 · 2021: 10 · 2020: 13 · 2019: 8 · 2018: 13 · 2017: 7 · 2016: 11 · 2015: 11 · 2014: 10 · 2013: 11 · 2012: 10
- FWCI
- 4.85
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Hepatology (publishedVersion)
- PubMed (publishedVersion)
- Max Planck Institute for Plasma Physics (submittedVersion)
- MPG.PuRe (Max Planck Society) (submittedVersion)
- Funders
- European Commission
- Fonds Wetenschappelijk Onderzoek
- Vlaamse regering
- Topics
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Clinical Biochemistry › Metabolism and Genetic Disorders
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Molecular Biology › Mitochondrial Function and Pathology
- Health Sciences › Medicine › Epidemiology › Autophagy in Disease and Therapy
- Keywords
- peroxisome deficiency, hepatocytes, PEX5, Zellweger syndrome, mitochondrial dysfunction, inner mitochondrial membrane, smooth endoplasmic reticulum, lipid droplets, lysosomes, hepatocyte hypertrophy, glycolysis, PPARα, ATP levels, oxidative stress
- MeSH terms
- Peroxisome-Targeting Signal 1 Receptor, Adenosine Triphosphate, Animals, Cells, Cultured, Disease Models, Animal, Endoplasmic Reticulum, Glucose, Liver, Mitochondria, Liver, Oxidation-Reduction, Zellweger Syndrome, Gene Expression, Receptors, Cytoplasmic and Nuclear, Mice, Knockout, Oxidative Stress, Peroxisomes, Hepatocytes, Mice
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