Mitochondrial dysfunction in congenital nephrotic syndrome

Citation
Ml. Solin et al., Mitochondrial dysfunction in congenital nephrotic syndrome, LAB INV, 80(8), 2000, pp. 1227-1232
Citations number
42
Categorie Soggetti
Medical Research General Topics
Journal title
LABORATORY INVESTIGATION
ISSN journal
00236837 → ACNP
Volume
80
Issue
8
Year of publication
2000
Pages
1227 - 1232
Database
ISI
SICI code
0023-6837(200008)80:8<1227:MDICNS>2.0.ZU;2-A
Abstract
The molecular mechanisms maintaining the kidney glomerular filtration barri er remain poorly understood. Recent evidence suggests that mitochondrial dy sfunction is a characteristic feature of kidney glomeruli in congenital nep hrotic syndrome of the Finnish type (CNF). Here we searched for detailed fu nctional evidence of mitochondrial lesion in CNF kidneys. We used histochem ical and immunohistochemical methods, quantitative measurement of mitochond rial DNA, and superoxide production to characterize the mitochondrial funct ion. The results unequivocally show down-regulation of mitochondria-encoded respiratory chain components, whereas the respective nuclearly encoded sub units were close to normal. These results give detailed evidence of distinc t mitochondrial dysfunction and of the resulting abnormal production of rea ctive oxygen species in CNF and suggest a critical role for mitochondria in maintaining the glomerular permeability barrier.