TRANSFER-RNA PROCESSING IN HUMAN MITOCHONDRIAL DISORDERS

Many human mitochondrial disorders are associated with mutations in tR NA genes or with deletions of regions containing tRNA genes, all of wh ich may be suspected to play a role in recognition by RNase P. Here we describe the analysis of five such mutations. The results presented h ere demonstrate that none of these mutations result in errors in RNase P function. Further studies of mutations in tRNAs need to be pursued to elucidate the identity elements for RNase P function in mammalian m itochondria.