Mitochondrial genome maintenance exonuclease 1 (EC 3.1.-.-)
1_MKMKL 6_ FQTIC 11_ RQLRS 16_ SKFSV 21_ ESAAL 26_ VAFST 31_ SSYSC 36_ GRKKK 41_ VNPYE 46_ EVDQE 51_ KYSNL 56_ VQSVL 61_ SSRGV 66_ AQTPG 71_ SVEED 76_ ALLCG 81_ PVSKH 86_ KLPNQ 91_ GEDRR 96_ VPQNW 101_ FPIFN 106_ PERSD 111_ KPNAS 116_ DPSVP 121_ LKIPL 126_ QRNVI 131_ PSVTR 136_ VLQQT 141_ MTKQQ 146_ VFLLE 151_ RWKQR 156_ MILEL 161_ GEDGF 166_ KEYTS 171_ NVFLQ 176_ GKRFH 181_ EALES 186_ ILSPQ 191_ ETLKE 196_ RDENL 201_ LKSGY 206_ IESVQ 211_ HILKD 216_ VSGVR 221_ ALESA 226_ VQHET 231_ LNYIG 236_ LLDCV 241_ AEYQG 246_ KLCVI 251_ DWKTS 256_ EKPKP 261_ FIQST 266_ FDNPL 271_ QVVAY 276_ MGAMN 281_ HDTNY 286_ SFQVQ 291_ CGLIV 296_ VAYKD 301_ GSPAH 306_ PHFMD 311_ AELCS 316_ QYWTK 321_ WLLRL 326_ EEYTE 331_ KKKNQ 336_NIQKP
1: Metal-dependent single-stranded DNA (ssDNA) exonuclease involved in mitochondrial genome maintenance. Has preference for 5'-3' exonuclease activity but is also capable of endonuclease activity on linear substrates. Necessary for maintenance of proper 7S DNA levels. Probably involved in mitochondrial DNA (mtDNA) repair, possibly via the processing of displaced DNA containing Okazaki fragments during RNA-primed DNA synthesis on the lagging strand or via processing of DNA flaps during long-patch base excision repair. Specifically binds 5-hydroxymethylcytosine (5hmC)-containing DNA in stem cells