Pseudouridylate synthase RPUSD4, mitochondrial (EC 5.4.99.-) (RNA pseudouridylate synthase domain-containing protein 4)
1_MAAPR 6_ WSASG 11_ PWIRG 16_ NGQGC 21_ GSLFT 26_ LVSKP 31_ FCAAA 36_ AASTA 41_ INAQR 46_ LAEKL 51_ RAQKR 56_ EQDTK 61_ KEPVS 66_ TNAVQ 71_ RRVQE 76_ IVRFT 81_ RQLQR 86_ VHPNV 91_ LAKAL 96_ TRGIL 101_ HQDKN 106_ LVVIN 111_ KPYGL 116_ PVHGG 121_ PGVQL 126_ CITDV 131_ LPILA 136_ KMLHG 141_ HKAEP 146_ LHLCH 151_ RLDKE 156_ TTGVM 161_ VLAWD 166_ KDMAH 171_ QVQEL 176_ FRTRQ 181_ VVKKY 186_ WAITV 191_ HVPMP 196_ SAGVV 201_ DIPIV 206_ EKEAQ 211_ GQQQH 216_ HKMTL 221_ SPSYR 226_ MDDGK 231_ MVKVR 236_ RSRNA 241_ QVAVT 246_ QYQVL 251_ SSTLS 256_ SALVE 261_ LQPIT 266_ GIKHQ 271_ LRVHL 276_ SFGLD 281_ CPILG 286_ DHKYS 291_ DWNRL 296_ APQKL 301_ SVGTL 306_ KKLGL 311_ EQSKA 316_ RYIPL 321_ HLHAR 326_ QLILP 331_ ALGSG 336_ KEELN 341_ LVCKL 346_ PRFFV 351_ HSLHR 356_ LRLEM 361_ PNEDQ 366_ NENNE 371_AKCLG
1: Catalyzes uridine to pseudouridine isomerization (pseudouridylation) of different mitochondrial RNA substrates (PubMed:27974379, PubMed:28082677). Acts on position 1397 in 16S mitochondrial ribosomal RNA (16S mt-rRNA) (PubMed:27974379). This modification is required for the assembly of 16S mt-rRNA into a functional mitochondrial ribosome (PubMed:27974379). As a component of a functional protein-RNA module, consisting of RCC1L, NGRN, RPUSD3, RPUSD4, TRUB2, FASTKD2 and 16S mt-rRNA, controls 16S mt-rRNA abundance and is required for intra-mitochondrial translation (PubMed:27667664). Acts on position 39 in mitochondrial tRNA(Phe) (PubMed:28082677). Also catalyzes pseudouridylation of mRNAs in nucleus: acts as a regulator of pre-mRNA splicing by mediating pseudouridylation of pre-mRNAs at locations associated with alternatively spliced regions (PubMed:35051350). Pseudouridylation of pre-mRNAs near splice sites directly regulates mRNA splicing and mRNA 3'-end processing (PubMed:35051350)