Abstract: Modified yeast cells comprising at least one mutation of the tryptophan 136 (W136), leucine 183 (L183), or leucine 247 (L247) codon of the mitochondrial ATP6 gene, responsible for NARP syndrome in humans and uses thereof for screening for medicaments that act against mitochondrial pathologies involving a deficiency in ATP production via the oxidative phosphorylation pathway, such as NARP syndrome.

Abstract: The invention relates to an industrial method for producing an interest heterologous RNA and to a system for carrying out said production method consisting (1) in transforming mitochondrions of yeast cells free of mitochondrial RNA with a mitochondrial transcription vector comprising at least one copy of the DNA encoding said interest heterologous RNA which are controlled by regulatory element(s) of the mitochondrial transcription and a reporter gene thereof or a fragment of said reporter gene, 2) identifying yeast transformants by incorporating the interest DNA into the mitochondrions thereof, (3) culturing the yeast mitochondrial transformants selected at the stage (2), (4) isolating the mitochondrions from the yeast mitochondrial transformants obtainable at stages (3) and in extracting and purifying the interest heterologous RNA from said mitochondrions.

Abstract: The present invention relates to means and methods for therapeutic intervention of mitochondrial disorders or diseases, in particular to a method for the treatment, prevention and/or amelioration of a disorder or disease correlated with mitochondrial dysfunction, a mitochondrial disorder or disease or a disorder or disease characterized by an altered OPA1 processing. Thereby, a pharmaceutically active amount of a compound capable of modulating the activity of an oligomeric complex comprising Afg3I1 and/or Afg3I2 or (a) variant(s) thereof is administered to a patient in need of medical intervention. The present invention also relates to the use of an oligomeric complex comprising Afg3I1 and/or Afg3I2 or (a) variant(s) thereof for the preparation of a pharmaceutical composition for the mentioned therapeutic intervention.

Abstract: Modified yeast cells comprising at least one mutation of the tryptophan 136 (W136), leucine 183 (L183), or leucine 247 (L247) codon of the mitochondrial ATP6 gene, responsible for NARP syndrome in humans and uses thereof for screening for medicaments that act against mitochondrial pathologies involving a deficiency in ATP production via the oxidative phosphorylation pathway, such as NARP syndrome.

Abstract: The invention relates to an industrial method for producing an interest heterologous RNA and to a system for carrying out said production method consisting (1) in transforming mitochondrions of yeast cells free of mitochondrial RNA with a mitochondrial transcription vector comprising at least one copy of the DNA encoding said interest heterologous RNA which are controlled by regulatory element(s) of the mitochondrial transcription and a reporter gene thereof or a fragment of said reporter gene, 2) identifying yeast transformants by incorporating the interest DNA into the mitochondrions thereof, (3) culturing the yeast mitochondrial transformants selected at the stage (2), (4) isolating the mitochondrions from the yeast mitochondrial transformants obtainable at stages (3) and in extracting and purifying the interest heterologous RNA from said mitochondrions.