Table 2. Genes involved in nucleic acid metabolism.

Gene deleted	Human ortholog	Wild-type function
RPO41		DNA-directed RNA polymerase, transcription from mitochondrial promoter
MGM101		DNA binding, mitochondrial genome maintenance
ABF2	HMG4	DNA binding, mitochondrial genome maintenance
RNR4	RRM2	Ribonucleotide reductase small subunit, DNA replication
SSQ1	HSPA9B	Mitochondrial heat shock protein 70, DNA dependent DNA replication
MSH1	MSH3	Mitochondrial DNA mismatch repair
MIP1		Mitochondrial DNA polymerase catalytic subunit, DNA- dependent DNA replication
FZO1		Mitochondrial integral membrane protein involved in mitochondrial fusion and maintenance of mitochondrial genome
MET7	FPGS	Tetrahydrofolylpolyglutamate synthase, required for methionine synthesis and for maintenance of mitochondrial DNA, involved in one-carbon compound metabolism
PET56	FLJ22578	Ribose methyltransferase for mitochondrial 21 S rRNA
NAM2	KIAA0028	Mitochondrial leucyl-tRNA synthetase, mRNA binding
SUV3	SUPV3L1	Mitochondrial ATP-dependent RNA helicase, RNA catabolism
HMI1		Mitochondrial inner membrane localized DNA helicase, maintenance of the mitochondrial genome
AEP3		Peripheral mitochondrial inner membrane protein, mRNA metabolism
MRH4	U5-100K	Mitochondrial RNA helicase, ribosome biogenesis