Table 1.

Gene products required for mtDNA replication and repair

Function	Gene	Protien	Size	Chromosome
Core replication	POLG	DNA polymerase	140 kDa	15q25
	POLG2	DNA polymerase accessory sutunit	55 kDa	17q23-24
	SSB	single stranded DNA binding protein	15 kDa	7q34
	PEO1 (Twinkle)	helicase	77 kDa	10q24
Replication and repair accessory factors	DNA ligase III	ligase	96kDa	17q11.2-12
	RNase H1	RNase	32 kDa	19p13.2
	Topo I	topoisomerase	67 kDa	8q24.3
	Topo IIIα	topoisomerase	112 kDa	17p12-11.2
	Fen-1	5′-3′ Flap endonuclease	43 kDa	11q12
	DNA2	5′-3′ DNA/RNA endonuclease/exonulclease	130 kDa	10q21.3-q22.1
	ExoG	5′-3′ Exonuclease	41 kDa	3p21.3
DNA repair proteins	UDG	Uracil DNA glycosylase	27.5 kDa	12q23-q24.1
	OGG1	8-oxo-dG glycosylase	38 kDa	3p26.2
	NTH1	Thymine glycol glycosylase	34 kDa	16p13.3
	MUTYH	glycosylase	60 kDa	1p34.3-p32.1
	NEIL1	Fapy glycosylase	44 kDa	15q4.2
	APE1	Ap endonuclease	35 kDa	14q11.2-q12
	APE2	Ap endonuclease	57 kDa	Xp11.22
Transcription	mtRNA Pol	Core RNA polymerase	150 kDa	19q13.3
	mtTFA	Transcription factor	24 kDa	10q21
	mtTFB1	Transcription factor	39 kDa	6q25.1-q25.3
	mtTFB2	Transcription factor	45 kDa	1q44