Table 2.

Nuclear genes involved in other mitochondrial functions causing Leigh and Leigh-like syndrome

Mitochondrial function	Associated nuclear genes
Mitochondrial DNA maintenance	FBXL4, POLG, POLG2, SUCLA2, SUCLG1, TWNK, SLC25A4, MPV17
Mitochondrial translation	GTPBP3, TRMU, EARS2, FARS2, IARS2, GFM1, GFM2, LRPPRC,TACO1, MTFMT, C12Orf65, MRPS34, MRPS39 (PTCD3), NARS2, TSFM
Mitochondrial dynamics	SLC25A46, DNM1L, MFN2, RRM2B
Mitochondrial material import/export	SLC25A19
Membrane phosphocomponents	SERAC1
Sulfur dioxygenase	ETHE1
AAA oligomeric +/− ATPase	CLPB, SPG7
RNA import	PNPT1
RNA-specific adenosine deaminase	ADAR, RNASEH1
Nuclear translocation system	RANBP2
Nuclear pore complex	NUP62
Manganese transportation	SLC39A8