The DOI matches its Crossref record; 93 of the 95 references it cites were found in a registry or an index.
Mitochondrial Alterations Caused by Defective Peroxisomal Biogenesis in a Mouse Model for Zellweger Syndrome (PEX5 Knockout Mouse)
Open accessAbstract
Zellweger syndrome (cerebro-hepato-renal syndrome) is the most severe form of the peroxisomal biogenesis disorders leading to early death of the affected children. To study the pathogenetic mechanisms causing organ dysfunctions in Zellweger syndrome, we have recently developed a knockout-mouse model by disrupting the PEX5 gene, encoding the targeting receptor for most peroxisomal matrix proteins (M Baes, P Gressens, E Baumgart, P Carmeliet, M Casteels, M Fransen, P Evrard, D Fahimi, PE Declercq, D Collen, PP van Veldhoven, GP Mannaerts: A mouse model for Zellweger syndrome. Nat Genet 1997, 17:49-57). In this study, we present evidence that the absence of functional peroxisomes, causing a general defect in peroxisomal metabolism, leads to proliferation of pleomorphic mitochondria with severe alterations of the mitochondrial ultrastructure, changes in the expression and activities of mitochondrial respiratory chain complexes, and an increase in the heterogeneity of the mitochondrial compartment in various organs and specific cell types (eg, liver, proximal tubules of the kidney, adrenal cortex, heart, skeletal and smooth muscle cells, neutrophils). The changes of mitochondrial respiratory chain enzymes are accompanied by a marked increase of mitochondrial manganese-superoxide dismutase, as revealed by in situ hybridization and immunocytochemistry, suggesting increased production of reactive oxygen species in altered mitochondria. This increased oxidative stress induced probably by defective peroxisomal antioxidant mechanisms combined with accumulation of lipid intermediates of peroxisomal beta-oxidation system could contribute significantly to the pathogenesis of multiple organ dysfunctions in Zellweger syndrome.
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A new pathway for mitochondrial quality control: Mitochondrial-derived vesicles
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“, liver [57,113], proximal tubules of the kidney [109,113], adrenal cortex [113], spinal cord [111,112], heart [113], and brain [62]) and cell types (e.”
Background
Metadata
- Authors
- Baumgart, E., Vanhorebeek, I., Grabenbauer, M., Borgers, M., Declercq, P. E., Fahimi, H. D., & Baes, M.
- Affiliations
- KU Leuven (BE)
- Heidelberg University (DE)
- Janssen (Belgium) (BE)
- Corresponding author
- Fahimi, H. Dariush
- Venue
- The American Journal of Pathology
- Publisher
- Elsevier BV
- Published
- 2001-10
- Volume
- 159
- Issue
- 4
- Pages
- 1477-1494
- ISSN
- 0002-9440 (ISSN-L)
- PMC
- PMC1850512
- OpenAlex
- W2151833520
- Cited by
- 210
- References
- 95
- Citations by year
- 2026: 4 · 2025: 11 · 2024: 10 · 2023: 5 · 2022: 11 · 2021: 8 · 2020: 10 · 2019: 12 · 2018: 11 · 2017: 6 · 2016: 11 · 2015: 8 · 2014: 10 · 2013: 10 · 2012: 8
- FWCI
- 3.28
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- American Journal Of Pathology (publishedVersion)
- PubMed Central (publishedVersion)
- PubMed (publishedVersion)
- Max Planck Institute for Plasma Physics (submittedVersion)
- MPG.PuRe (Max Planck Society) (submittedVersion)
- Funders
- Deutsche Forschungsgemeinschaft (Ba 1155/1-4, SFB 601-B1)
- 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
- Life Sciences › Biochemistry, Genetics and Molecular Biology › Cell Biology › Autophagy in Disease and Therapy
- Keywords
- Zellweger syndrome, peroxisome biogenesis disorders, mitochondrial ultrastructure, respiratory chain complexes, manganese superoxide dismutase, reactive oxygen species, oxidative stress, peroxisomal beta-oxidation, lipid intermediates, organ dysfunction
- MeSH terms
- Peroxisome-Targeting Signal 1 Receptor, Adenosine Triphosphate, Animals, Autophagy, Blood Cells, Electron Transport Complex IV, Cytoplasm, Disease Models, Animal, Electron Transport, Mitochondria, Mitochondria, Heart, Mitochondria, Liver, NADH, NADPH Oxidoreductases, Oxidation-Reduction, Superoxide Dismutase, Tissue Distribution, Zellweger Syndrome, Reactive Oxygen Species, Receptors, Cytoplasmic and Nuclear, Mice, Knockout, Peroxisomes, Hepatocytes, Electron Transport Complex I, Mice
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