Persulfide dioxygenase ETHE1, mitochondrial (EC 1.13.11.18) (Ethylmalonic encephalopathy protein 1) (Hepatoma subtracted clone one protein) (Sulfur dioxygenase ETHE1)
1_MAEAV 6_ LRVAR 11_ RQLSQ 16_ RGGSG 21_ APILL 26_ RQMFE 31_ PVSCT 36_ FTYLL 41_ GDRES 46_ REAVL 51_ IDPVL 56_ ETAPR 61_ DAQLI 66_ KELGL 71_ RLLYA 76_ VNTHC 81_ HADHI 86_ TGSGL 91_ LRSLL 96_ PGCQS 101_ VISRL 106_ SGAQA 111_ DLHIE 116_ DGDSI 121_ RFGRF 126_ ALETR 131_ ASPGH 136_ TPGCV 141_ TFVLN 146_ DHSMA 151_ FTGDA 156_ LLIRG 161_ CGRTD 166_ FQQGC 171_ AKTLY 176_ HSVHE 181_ KIFTL 186_ PGDCL 191_ IYPAH 196_ DYHGF 201_ TVSTV 206_ EEERT 211_ LNPRL 216_ TLSCE 221_ EFVKI 226_ MGNLN 231_ LPKPQ 236_ QIDFA 241_ VPANM 246_RCGVQ
1: Sulfur dioxygenase that plays an essential role in hydrogen sulfide catabolism in the mitochondrial matrix. Hydrogen sulfide (H(2)S) is first oxidized by SQRDL, giving rise to cysteine persulfide residues. ETHE1 consumes molecular oxygen to catalyze the oxidation of the persulfide, once it has been transferred to a thiophilic acceptor, such as glutathione (R-SSH). Plays an important role in metabolic homeostasis in mitochondria by metabolizing hydrogen sulfide and preventing the accumulation of supraphysiological H(2)S levels that have toxic effects, due to the inhibition of cytochrome c oxidase. First described as a protein that can shuttle between the nucleus and the cytoplasm and suppress p53-induced apoptosis by sequestering the transcription factor RELA/NFKB3 in the cytoplasm and preventing its accumulation in the nucleus (PubMed:12398897)