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Detection of mitochondrial defects by laser fluorimetry
W S Kunz1, K Winkler, A V Kuznetsov
1Klinik für Neurologie, Universitätsklinikum Magdeburg, Germany.
Molecular and Cellular Biochemistry
|October 6, 1997
Summary
Chronic progressive external ophthalmoplegia patients show altered mitochondrial function. Muscle fibers exhibit elevated NAD(P)H redox states and increased autofluorescence, indicating more, but partially inhibited, mitochondria.
Area of Science:
- Mitochondrial Medicine
- Neuromuscular Disorders
- Biochemistry
Background:
- Chronic progressive external ophthalmoplegia (CPEO) is a mitochondrial disorder.
- Mitochondrial DNA (mtDNA) deletions are a common cause of CPEO.
- Skeletal muscle dysfunction is a hallmark of CPEO.