A review in German language is given on recent advances in studying mo
lecular and genetic aspects of mitochondrial inheritance. The first pa
rt of this review deals with structure and form of mitochondrial DNA (
mitDNA) and the complete mitDNA sequence of several species; mitDNA re
arrangements and mitochondrial plasmids are also described. The topics
of the following chapters are: replication and coding capacity of mit
DNA, cis- and trans-splicing and RNA editing as well as the processes
of tRNA-trafficking and of protein import into mitochondria. The surve
y on mitochondrial genetics (mainly in yeast, but also in Neurospora a
nd Paramecium) starts with the characterization of different classes o
f mutants, then deals with the recombination of mitochondrial genes as
well as with the genetic methods for mapping mitochondrial gene mutat
ions, and describes the present status of the physical mapping of mito
chondrial genomes of several species. Finally mitochondrial mutations
in man are shown to be the molecular basis of several hereditary disea
ses.