Requirement for the yeast gene LON in intramitochondrial proteolysis and maintenance of respiration - PubMed (original) (raw)

. 1994 Apr 8;264(5156):273-6.

doi: 10.1126/science.8146662.

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Requirement for the yeast gene LON in intramitochondrial proteolysis and maintenance of respiration

C K Suzuki et al. Science. 1994.

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Abstract

The role of protein degradation in mitochondrial homeostasis was explored by cloning of a gene from Saccharomyces cerevisiae that encodes a protein resembling the adenosine triphosphate (ATP)-dependent bacterial protease Lon. The predicted yeast protein has a typical mitochondrial matrix-targeting sequence at its amino terminus. Yeast cells lacking a functional LON gene contained a nonfunctional mitochondrial genome, were respiratory-deficient, and lacked an ATP-dependent proteolytic activity present in the mitochondria of Lon+ cells. Lon- cells were also impaired in their ability to catalyze the energy-dependent degradation of several mitochondrial matrix proteins and they accumulated electron-dense inclusions in their mitochondrial matrix.

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