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The uniqueness of the mitochondrial genome presents a number of obstacles to the successful use of gene therapy for the treatment of mitochondrial DNA disease. A new study shows that the effects of a pathogenic mutation in a human mitochondrial gene can be rectified by expressing an engineered wildtype copy of the gene in the nucleus.
The identification of a modifier of the tubby mutation, associated with deafness, neuronal degeneration and obesity, offers new insights into the molecular mechanisms of sound perception and its disorders.
A model for direct regulation of an entire genetic network by an organ-identity gene has been proposed. The relative affinities of the organ-identity transcription factor for different target sites control the timing of expression of downstream genes during development.
Using comparative sequence analyses, we can identify proteins that may have been subject to positive darwinian selection. To test these statistical results, it is important to develop functional assays and identify amino-acid changes that are responsible for the adaptation of organisms to specific environments. One of the two duplicated pancreatic ribonuclease genes of a leaf-eating colobine monkey, douc langur, is now shown to have adapted to digest bacterial RNAs in the monkey's foregut.
Mitochondrial signaling cascades have been implicated in the activation of programmed cell death and, more recently, control of cell proliferation. A nuclear gene encoding a mitochondrial Krebs-cycle protein, fumarate hydratase, is now shown to act as a major tumor-suppressor gene.