Region-Specific Defects of Respiratory Capacities in the Ndufs4(KO) Mouse Brain.

ABSTRACT:

Background

Lack of NDUFS4, a subunit of mitochondrial complex I (NADH:ubiquinone oxidoreductase), causes Leigh syndrome (LS), a progressive encephalomyopathy. Knocking out Ndufs4, either systemically or in brain only, elicits LS in mice. In patients as well as in KO mice distinct regions of the brain degenerate while surrounding tissue survives despite systemic complex I dysfunction. For the understanding of disease etiology and ultimately for the development of rationale treatments for LS, it appears important to uncover the mechanisms that govern focal neurodegeneration.

Results

Here we used the Ndufs4(KO) mouse to investigate whether regional and temporal differences in respiratory capacity of the brain could be correlated with neurodegeneration. In the KO the respiratory

SUBMITTER: Kayser EB 

PROVIDER: S-EPMC4732614 | biostudies-literature | 2016

REPOSITORIES: biostudies-literature