This review attempts to summarize our present state of knowledge of mitocho
ndria in relation to a number of areas of biology, and to indicate where fu
ture research might be directed. In the evolution of eukaryotic cells mitoc
hondria have for a long time played a prominent role. Nowadays their integr
ation into many activities of a cell, and their dynamic behavior as subcell
ular organelles within a cell and during cell division are a major focus of
attention. The crystal structures of the major complexes of the electron t
ransport chain (except complex I) have been established, permitting increas
ingly detailed analyses of the important mechanism of proton pumping couple
d to electron transport. The mitochondrial genome and its replication and e
xpression are beginning to be understood in considerable detail, but more q
uestions remain with regard to mutations and their repair, and the segregat
ion of the mtDNA in oogenesis and development. Much emphasis and a large ef
fort have recently been devoted to understand the role of mitochondria in p
rogrammed cell death (apoptosis). The understanding of their central role i
n mitochondrial diseases is a major achievement of the past decade. Finally
, various drugs have traditionally played a part in understanding biochemic
al mechanisms within mitochondria; the repertoire of drugs with novel and i
nteresting targets is expanding. (C) 2001 Published by Elsevier Science B.V
.