In the medical literature the term 'mitochondrial disorders' is to a large extent applied to the
clinical syndromes associated with abnormalities of the common final pathway of …

This review focuses on mutations of mitochondrial DNA (mtDNA) which are an important
cause of mitochondrial disorders in humans and are also associated with common …

A variable combination of developmental delay, retinitis pigmentosa, dementia, seizures,
ataxia, proximal neurogenic muscle weakness, and sensory neuropathy occurred in four …

Studies of diseases caused by mitochondrial DNA mutations suggest that a variety of
degenerative processes may be associated with defects in oxidative phosphorylation …

Abstract The 16,775 base-pair mitochondrial genome of the white Leghorn chicken has
been cloned and sequenced. The avian genome encodes the same set of genes (13 …

Diabetes mellitus (DM) is one of the most common chronic disorders of children and adults.
Several reports have suggested an increased incidence of maternal transmission in some …

The role of somatic mitochondrial DNA (mtDNA) damage in human aging and progressive
diseases of oxidative phosphorylation (OXPHOS) was examined by quantitating the …

Family studies of diabetes mellitus (DM) show that patients are more likely to have affected
mothers than affected fathers. Since the inheritance of mitochondrial (mtDNA), unlike …

Mitrochondrial diseases are uniquely interesting from a genetic point of view because
mitochondria contain their own DNA (mtDNA) and are capable of synthesizing a small but …

We studied two related infants with a fatal mitochondrial disease, affecting muscle in one
and liver in the other. Quantitative analysis revealed a severe depletion of mtDNA in affected …