Bayat et al. [40] described mutations in MARS2 encoding the mitochondrial methionyl-tRNA synthetase (mt-MetRS), which cause autosomal recessive spastic ataxia with leukoencephalopathy (ARSAL or spastic ataxia 3, OMIM#611390) in humans and neurodegeneration in flies. The gene discussed is MARS2; the disease is spastic ataxia 3.