PMID:21910628

Human mitochondrial tRNAs: biogenesis, function, structural aspects, and diseases.

The 22 mtDNA-encoded tRNAs are used by the mitochondrial protein synthesis machinery to translate the 13 mtDNA-encoded mRNAs.

"These mRNAs are translated by the mitochondrial protein synthesis machinery, which uses the 22 species of mitochondrial tRNAs (mt tRNAs) encoded by mtDNA."
mt tRNA genes are frequent sites of pathogenic point mutations.

"The genes encoding mt tRNAs are highly susceptible to point mutations, which are a primary cause of mitochondrial dysfunction and are associated with a wide range of pathologies."

PMID:7219534

Sequence and organization of the human mitochondrial genome.

The human mitochondrial genome encodes 22 tRNAs.

"The genes for the 12S and 16S rRNAs, 22 tRNAs, cytochrome c oxidase subunits I, II and III, ATPase subunit 6, cytochrome b and eight other predicted protein coding genes have been located."

PMID:17132941

Human mitochondrial diseases associated with tRNA wobble modification deficiency.

tRNA-Lys normally reads AAA and AAG; the MERRF mutant lacking the wobble modification reads neither.

"The MERRF mt tRNA(Lys) lacking the wobble modification cannot translate either of its codons (AAA and AAG)"