| Mitochondrial dysfunction in congenital nephrotic syndrome. | |
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MedLine Citation:
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PMID: 10950113 Owner: NLM Status: MEDLINE |
Abstract/OtherAbstract:
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The molecular mechanisms maintaining the kidney glomerular filtration barrier remain poorly understood. Recent evidence suggests that mitochondrial dysfunction is a characteristic feature of kidney glomeruli in congenital nephrotic syndrome of the Finnish type (CNF). Here we searched for detailed functional evidence of mitochondrial lesion in CNF kidneys. We used histochemical and immunohistochemical methods, quantitative measurement of mitochondrial DNA, and superoxide production to characterize the mitochondrial function. The results unequivocally show down-regulation of mitochondria-encoded respiratory chain components, whereas the respective nuclearly encoded subunits were close to normal. These results give detailed evidence of distinct mitochondrial dysfunction and of the resulting abnormal production of reactive oxygen species in CNF and suggest a critical role for mitochondria in maintaining the glomerular permeability barrier. |
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Authors:
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M L Solin; S Pitkänen; J W Taanman; H Holthöfer |
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Publication Detail:
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Type: Journal Article |
Journal Detail:
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Title: Laboratory investigation; a journal of technical methods and pathology Volume: 80 ISSN: 0023-6837 ISO Abbreviation: Lab. Invest. Publication Date: 2000 Aug |
Date Detail:
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Created Date: 2000-08-31 Completed Date: 2000-08-31 Revised Date: 2004-11-17 |
Medline Journal Info:
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Nlm Unique ID: 0376617 Medline TA: Lab Invest Country: UNITED STATES |
Other Details:
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Languages: eng Pagination: 1227-32 Citation Subset: IM |
Affiliation:
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Haartman Institute, Division of Bacteriology and Immunology, University of Helsinki, Finland. |
Export Citation:
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| MeSH Terms | |
Descriptor/Qualifier:
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DNA, Mitochondrial
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genetics Electron Transport Electron Transport Complex IV / metabolism Humans Mitochondria / metabolism, physiology* NADH Dehydrogenase / metabolism Nephrotic Syndrome / congenital*, metabolism, physiopathology* Reactive Oxygen Species Succinate Cytochrome c Oxidoreductase / metabolism Superoxides / metabolism |
| Chemical | |
Reg. No./Substance:
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0/DNA, Mitochondrial; 0/Reactive Oxygen Species; 11062-77-4/Superoxides; EC 1.-/Succinate Cytochrome c Oxidoreductase; EC 1.6.99.3/NADH Dehydrogenase; EC 1.9.3.1/Electron Transport Complex IV |
From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine
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