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Related Experiment Videos

Mitochondrial dysfunction in congenital nephrotic syndrome.

M L Solin1, S Pitkänen, J W Taanman

  • 1Haartman Institute, Division of Bacteriology and Immunology, University of Helsinki, Finland.

Laboratory Investigation; a Journal of Technical Methods and Pathology
|August 19, 2000
PubMed
Summary

Mitochondrial dysfunction causes abnormal reactive oxygen species production in congenital nephrotic syndrome of the Finnish type (CNF). This dysfunction plays a critical role in maintaining the kidney glomerular filtration barrier.

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Area of Science:

  • Nephrology
  • Mitochondrial Biology
  • Molecular Medicine

Background:

  • The molecular mechanisms governing the kidney glomerular filtration barrier are not fully understood.
  • Mitochondrial dysfunction is increasingly recognized as a feature in congenital nephrotic syndrome of the Finnish type (CNF).

Purpose of the Study:

  • To investigate detailed functional evidence of mitochondrial damage in CNF kidneys.
  • To elucidate the role of mitochondria in maintaining glomerular filtration.

Main Methods:

  • Histochemical and immunohistochemical analyses were employed.
  • Quantitative measurement of mitochondrial DNA and superoxide production was performed.

Main Results:

  • A significant down-regulation of mitochondria-encoded respiratory chain components was observed.

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  • Nuclear-encoded subunits of the respiratory chain remained largely normal.
  • Abnormal production of reactive oxygen species was detected in CNF kidneys.

    • Conclusions:

    • Distinct mitochondrial dysfunction is evident in CNF.
    • Mitochondria play a critical role in maintaining glomerular permeability.
    • Abnormal reactive oxygen species production contributes to CNF pathogenesis.