Alanine–tRNA ligase, mitochondrial, also known as alanyl-tRNA synthetase 2 (AARS2), is an enzyme that in humans is encoded by the AARS2 gene. Like the cytosolic variety, AARS2, this enzyme functions as an alanine–tRNA ligase, which means it attaches the amino acid alanine to the corresponding transfer RNA (tRNAAla) in preparation of RNA-to-protein translation.

Clinical relevance Mutations in the AARS2 gene result in infantile mitochondrial cardiomyopathies. Progressive leukoencephalopathy with Ovarian Failure (LKENP).

References

Further reading

See also Aminoacyl tRNA synthetase

External links

CureARS non profit organisation supporting families affected by ARS mutations, including AARS2.