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. Author manuscript; available in PMC: 2015 Aug 23.
Published in final edited form as: J Cell Biochem. 2015 Aug;116(8):1490–1499. doi: 10.1002/jcb.25103

Table 1.

Nuclear and Mitochondrial Human DNA Glycosylases

FAMILY Fold ISOFORMS
GLYCOSYLASE Nuclear Mitochondrial SUBSTRATES

UDG UNG UNG2 UNG1 U, 5-FU & ssDNA, dsDNA
TDG TDG NF T:G, U:G, εC:G, 5-FU, Tg:G & dsDNA>>ssDNA
SMUG1 SMUG1 NF U, 5-hmU, 5-hmC, 5-FU & ssDNA, dsDNA

AAG AAG/MPG A,B,C A, B 3-meA, 7-meG, 1-meG, Hx, U, εG, εA & ssDNA, dsDNA

Helix-hairpin-helix NTHL1 NTHL1 NTHL1 Tg, 5-hC, 5-hU, Fapy lesions & ssDNA, dsDNA
OGG1 1a 1b, 1c; 2a – 2e 8-oxoG, Fapy lesions & dsDNA
MUTYH β, γ α A:8-oxoG, A:G, and A:C, 2-OHA:G & dsDNA
MBD4 MBD4 NF T:G, U:G, 5-MeC, halogenated pyrimidines, 5-FU, Tg:G & dsDNA

Fpg/Nei Helix-two turns-helix NEIL1 NEIL1 NEIL1 Sp, Gh, Tg, DHU, 5-OHU, 5-OHC, DHT, FapyG, FapyA & dsDNA>bubble, bulge, fork>ssDNA
NEIL2 NEIL2 NEIL2 Sp, Gh, DHT, DHU, 5-OHU, 5-OHC & ssDNA>bubble, bulge, fork>dsDNA
NEIL3 NEIL3 NF Sp, Gh, FapyG, FapyA, MeFapyG, DHU, DHT, 5-OHU, 5-OHC, Tg & ssDNA>dsDNA

Isoforms specific for the mitochondria or the nucleus have been described so far for AAG, OGG1 and MUTYH. The substrate preferences for each glycosylase listed in Table 1 have been reviewed extensively [Brooks et al., 2013; Jacobs and Schar, 2012; Liu et al., 2013; Prakash et al., 2012]. NF, not found in the mitochondria.