FL-PGC-1α -/- mice have a symptomatology corresponding with mitochondrial encephalopathy.
The behavioral alterations intriguingly do not progress with age.
The alterations do not associate with retinal or spinal cord involvement.
The histopathology of skeletal muscle demonstrates only mild myopathic changes.
CNS-specific isoforms are the predominant PGC-1α mRNAs in the murine brain.