Very recently, following the same strategy used for YARS2, several missense variants in DARS2 gene were modeled in yeast, using the DARS2 orthologous gene MSD1. DARS2 mutations were found in patients affected by Leukoencephalopathy with brainstem and spinal cord involvement and lactate elevation (LBSL). Here, DARS2 is linked to Leukoencephalopathy.