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Altered mitochondrial function in canine ceroid-lipofuscinosis

A N Siakotos¹, P S Blair, J D Savill

¹Department of Pathology-Laboratory Medicine, Indiana University School of Medicine, Indianapolis 46202-5122, USA.

Neurochemical Research

|August 5, 1998

Summary

Neuronal ceroid-lipofuscinoses (NCL) involve impaired mitochondrial function, evidenced by altered oxidative phosphorylation in affected dogs. This dysfunction may contribute to the neurodegenerative disease pathology.

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Area of Science:

Biochemistry
Neuroscience
Genetics

Background:

Neuronal ceroid-lipofuscinoses (NCL) are inherited neurodegenerative diseases.
NCL involves dementia, neuronal atrophy, and premature death.
Massive accumulation of mitochondrial ATP synthase subunit c protein occurs in NCL.

Purpose of the Study:

To investigate mitochondrial function in NCL.
To determine if oxidative phosphorylation is altered in NCL.

Main Methods:

Studied liver mitochondria from English setters with NCL.
Measured ADP/O ratios and ADP-stimulated respiration rates.
Assessed mitochondrial ADP content.

Main Results:

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ADP/O ratios were depressed in affected and carrier dogs, indicating uncoupled oxidative phosphorylation.

ADP-stimulated respiration rates were elevated, particularly in carriers.

Mitochondrial ADP content increased, suggesting enhanced ADP uptake.

Conclusions:

Mitochondrial dysfunction, specifically altered oxidative phosphorylation, is present in NCL.
Elevated respiration may be a compensatory response to reduced efficiency.
Impaired mitochondrial function likely contributes to NCL pathology.