A proposed consensus panel of organisms for determining evolutionary conservation of mt-tRNA point mutations

► Assigning pathogenicity to mt-tRNA variants requires multiple veins of evidence, including evolutionary conservation; however, there is no “standard sequence panel”. ► Species used for conservation assessment previously, noting alarming variation. ► Whether a panel with a single sequence from each organism can be used to assess conservation, given intra species variation. ► We set out robust criteria for sequence selection and suggest a “standard sequence panel”. ► Aid decisions on when to carry out functional studies upon identification of novel mutations and improve consistency of mt-tRNA mutation reporting.