The understanding of mitochondrial biology and, subsquently, the role of mi
tochondrial pathology in human disease has increased exponentially over the
past 30 years. As insight has increased, so attention has begun to shift t
o the possibilities for treating mitochondrially based disorders. There are
a number of archetypal mitochondrial diseases, each associated with specif
ic mitochondrial DNA mutations, deletions, or depletions. In addition there
are a number of disorders, mainly neurodegenerative in nature, in which mi
tochondrial dysfunction appears to play a pivotal role. Mitochondrial struc
ture and function are discussed. Treatment of the archetypal mitochondrial
disorders and other neurogenerative conditions is reviewed, with specific e
mphasis on the prospects for neuroprotection. (C) 1998 Wiley-Liss, Inc.