Mitochondrial Genes and Translation Products

A putative precursor of rat liver mitochondrial malate dehydrogenase

“…Mitochondria have their own genetic system but the majority of mitochondrial proteins are coded by nuclear DNA, synthesized on cytosolic ribosomes and imported into mitochondria [ 1,2]. Little is known of the mechanism of import [3-61. There is some evidence that the mature form or subunits of the mitochondrial matrix proteins aspartate aminotransferase [6-91 and malate dehydrogenase (EC 1 .l .1.37) [6,1 O-l 21 can be taken up into the matrix of isolated mitochondria.…”

Genetics of the mammalian oxidative phosphorylation system: characterization of a new oligomycin-resistant Chinese hamster ovary cell line.

“…Several components of the mitochondrial oxidative phosphorylation system (e.g., the ATPase, cytochrome c oxidase, and coenzyme QH2-cytochrome c reductase) require the synthesis and assembly of gene products coded by both the nuclear and the mitochondrial genomes (1,5,41,43,49). Mutations affecting various components of the oxidative phosphorylation system in lower eucaryotes and bacteria have contributed to our understanding of the synthesis and assembly of these gene products and have permitted mapping the genes for several of these products (for reviews, see references 5,15,41,49). These mutants have also been useful in studying the transmission, recombination, and segregation of genes coded by the mitochondrial DNA (for reviews, see references 3, 23).…”

The mitochondrially made subunit 2 of Neurospora crassa cytochrome aa3 is synthesized as a precursor protein

“…Biogenesis of mitochondria requires the coordinate activity of two genetic systems: most of the mitochondrial proteins are synthesized in the cytoplasm on mRNAs transcribed from nuclear genes, whereas only a limited number of polypeptides is coded for by the mitochondrial genome and synthesized inside the organelle [1,2]. Most of the mitochondrially synthesized proteins are located in the mitochondrial inner membrane.…”