ID MTEF3_MOUSE Reviewed; 412 AA. AC Q8R3J4; Q9CSV1; Q9CWM7; DT 31-OCT-2006, integrated into UniProtKB/Swiss-Prot. DT 01-JUN-2002, sequence version 1. DT 16-JAN-2019, entry version 100. DE RecName: Full=Transcription termination factor 3, mitochondrial; DE AltName: Full=Mitochondrial transcription termination factor 3; DE Short=mTERF3; DE AltName: Full=mTERF domain-containing protein 1, mitochondrial; DE Flags: Precursor; GN Name=Mterf3; Synonyms=Mterfd1; OrderedLocusNames=CGI-12; OS Mus musculus (Mouse). OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; OC Mammalia; Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; OC Muroidea; Muridae; Murinae; Mus; Mus. OX NCBI_TaxID=10090; RN [1] RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. RC STRAIN=C57BL/6J; TISSUE=Embryonic stem cell; RX PubMed=16141072; DOI=10.1126/science.1112014; RA Carninci P., Kasukawa T., Katayama S., Gough J., Frith M.C., Maeda N., RA Oyama R., Ravasi T., Lenhard B., Wells C., Kodzius R., Shimokawa K., RA Bajic V.B., Brenner S.E., Batalov S., Forrest A.R., Zavolan M., RA Davis M.J., Wilming L.G., Aidinis V., Allen J.E., RA Ambesi-Impiombato A., Apweiler R., Aturaliya R.N., Bailey T.L., RA Bansal M., Baxter L., Beisel K.W., Bersano T., Bono H., Chalk A.M., RA Chiu K.P., Choudhary V., Christoffels A., Clutterbuck D.R., RA Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., Della Gatta G., RA di Bernardo D., Down T., Engstrom P., Fagiolini M., Faulkner G., RA Fletcher C.F., Fukushima T., Furuno M., Futaki S., Gariboldi M., RA Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., RA Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., RA Hill D., Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., RA Jakt M., Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., RA Kitano H., Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., RA Kurochkin I.V., Lareau L.F., Lazarevic D., Lipovich L., Liu J., RA Liuni S., McWilliam S., Madan Babu M., Madera M., Marchionni L., RA Matsuda H., Matsuzawa S., Miki H., Mignone F., Miyake S., Morris K., RA Mottagui-Tabar S., Mulder N., Nakano N., Nakauchi H., Ng P., RA Nilsson R., Nishiguchi S., Nishikawa S., Nori F., Ohara O., RA Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., Pesole G., RA Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., Ringwald M., RA Rost B., Ruan Y., Salzberg S.L., Sandelin A., Schneider C., RA Schoenbach C., Sekiguchi K., Semple C.A., Seno S., Sessa L., Sheng Y., RA Shibata Y., Shimada H., Shimada K., Silva D., Sinclair B., RA Sperling S., Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., RA Tammoja K., Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., RA Ueda H.R., van Nimwegen E., Verardo R., Wei C.L., Yagi K., RA Yamanishi H., Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., RA Grimmond S.M., Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., RA Wahlestedt C., Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., RA Fukuda S., Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., RA Iida J., Imamura K., Itoh M., Kato T., Kawaji H., Kawagashira N., RA Kawashima T., Kojima M., Kondo S., Konno H., Nakano K., Ninomiya N., RA Nishio T., Okada M., Plessy C., Shibata K., Shiraki T., Suzuki S., RA Tagami M., Waki K., Watahiki A., Okamura-Oho Y., Suzuki H., Kawai J., RA Hayashizaki Y.; RT "The transcriptional landscape of the mammalian genome."; RL Science 309:1559-1563(2005). RN [2] RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA]. RC STRAIN=C57BL/6J, and FVB/N-3; TISSUE=Kidney, and Mammary tumor; RX PubMed=15489334; DOI=10.1101/gr.2596504; RG The MGC Project Team; RT "The status, quality, and expansion of the NIH full-length cDNA RT project: the Mammalian Gene Collection (MGC)."; RL Genome Res. 14:2121-2127(2004). RN [3] RP DISRUPTION PHENOTYPE, AND FUNCTION. RX PubMed=17662942; DOI=10.1016/j.cell.2007.05.046; RA Park C.B., Asin-Cayuela J., Camara Y., Shi Y., Pellegrini M., RA Gaspari M., Wibom R., Hultenby K., Erdjument-Bromage H., Tempst P., RA Falkenberg M., Gustafsson C.M., Larsson N.G.; RT "MTERF3 is a negative regulator of mammalian mtDNA transcription."; RL Cell 130:273-285(2007). RN [4] RP FUNCTION. RX PubMed=23300484; DOI=10.1371/journal.pgen.1003178; RA Wredenberg A., Lagouge M., Bratic A., Metodiev M.D., Spaahr H., RA Mourier A., Freyer C., Ruzzenente B., Tain L., Groenke S., Baggio F., RA Kukat C., Kremmer E., Wibom R., Polosa P.L., Habermann B., RA Partridge L., Park C.B., Larsson N.G.; RT "MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates RT and mammals."; RL PLoS Genet. 9:E1003178-E1003178(2013). CC -!- FUNCTION: Binds promoter DNA and regulates initiation of CC transcription (By similarity). Required for normal mitochondrial CC transcription and translation, and for normal assembly of CC mitochondrial respiratory complexes (PubMed:17662942, CC PubMed:23300484). Required for normal mitochondrial function CC (PubMed:17662942, PubMed:23300484). Maintains 16S rRNA levels and CC functions in mitochondrial ribosome assembly by regulating the CC biogenesis of the 39S ribosomal subunit (PubMed:17662942, CC PubMed:23300484). {ECO:0000250|UniProtKB:Q96E29, CC ECO:0000269|PubMed:17662942, ECO:0000269|PubMed:23300484}. CC -!- SUBCELLULAR LOCATION: Mitochondrion CC {ECO:0000250|UniProtKB:Q96E29}. CC -!- DOMAIN: Contains seven structural repeats of about 35 residues, CC where each repeat contains three helices. The repeats form a CC superhelical structure with a solenoid shape. CC {ECO:0000250|UniProtKB:Q96E29}. CC -!- DISRUPTION PHENOTYPE: Embryonic lethality, due to severe CC mitochondrial dysfunction. Embryos are much smaller than normal CC and none survive past E10.5. {ECO:0000269|PubMed:17662942}. CC -!- SIMILARITY: Belongs to the mTERF family. {ECO:0000305}. CC --------------------------------------------------------------------------- CC Copyrighted by the UniProt Consortium, see https://www.uniprot.org/terms CC Distributed under the Creative Commons Attribution (CC BY 4.0) License CC --------------------------------------------------------------------------- DR EMBL; AK010525; BAB27006.1; -; mRNA. DR EMBL; AK011934; BAB27924.3; -; mRNA. DR EMBL; BC025173; AAH25173.1; -; mRNA. DR EMBL; BC085282; AAH85282.1; -; mRNA. DR CCDS; CCDS26611.1; -. DR RefSeq; NP_079823.2; NM_025547.3. DR RefSeq; XP_006517384.1; XM_006517321.2. DR RefSeq; XP_011242850.1; XM_011244548.2. DR RefSeq; XP_017171052.1; XM_017315563.1. DR UniGene; Mm.347939; -. DR ProteinModelPortal; Q8R3J4; -. DR SMR; Q8R3J4; -. DR STRING; 10090.ENSMUSP00000021991; -. DR iPTMnet; Q8R3J4; -. DR PhosphoSitePlus; Q8R3J4; -. DR EPD; Q8R3J4; -. DR MaxQB; Q8R3J4; -. DR PaxDb; Q8R3J4; -. DR PeptideAtlas; Q8R3J4; -. DR PRIDE; Q8R3J4; -. DR DNASU; 66410; -. DR Ensembl; ENSMUST00000021991; ENSMUSP00000021991; ENSMUSG00000021519. DR GeneID; 66410; -. DR KEGG; mmu:66410; -. DR UCSC; uc007qzw.2; mouse. DR CTD; 51001; -. DR MGI; MGI:1913660; Mterf3. DR eggNOG; KOG1267; Eukaryota. DR eggNOG; ENOG410XT49; LUCA. DR GeneTree; ENSGT00390000005801; -. DR HOGENOM; HOG000044443; -. DR HOVERGEN; HBG062577; -. DR InParanoid; Q8R3J4; -. DR KO; K15032; -. DR OMA; NNFYFVR; -. DR OrthoDB; 1294087at2759; -. DR PhylomeDB; Q8R3J4; -. DR TreeFam; TF317943; -. DR Reactome; R-MMU-5205685; Pink/Parkin Mediated Mitophagy. DR PRO; PR:Q8R3J4; -. DR Proteomes; UP000000589; Chromosome 13. DR Bgee; ENSMUSG00000021519; Expressed in 288 organ(s), highest expression level in islet of Langerhans. DR CleanEx; MM_MTERFD1; -. DR ExpressionAtlas; Q8R3J4; baseline and differential. DR Genevisible; Q8R3J4; MM. DR GO; GO:0005739; C:mitochondrion; HDA:MGI. DR GO; GO:0005654; C:nucleoplasm; ISO:MGI. DR GO; GO:0003690; F:double-stranded DNA binding; IEA:InterPro. DR GO; GO:0044212; F:transcription regulatory region DNA binding; ISS:UniProtKB. DR GO; GO:0061668; P:mitochondrial ribosome assembly; IBA:GO_Central. DR GO; GO:0006390; P:mitochondrial transcription; IBA:GO_Central. DR GO; GO:0032543; P:mitochondrial translation; IBA:GO_Central. DR GO; GO:0045892; P:negative regulation of transcription, DNA-templated; IMP:UniProtKB. DR Gene3D; 1.25.70.10; -; 1. DR InterPro; IPR003690; MTERF. DR InterPro; IPR038538; MTERF_sf. DR Pfam; PF02536; mTERF; 1. DR SMART; SM00733; Mterf; 6. PE 2: Evidence at transcript level; KW Complete proteome; DNA-binding; Mitochondrion; Reference proteome; KW Ribosome biogenesis; Transcription; Transcription regulation; KW Transit peptide. FT TRANSIT 1 67 Mitochondrion. FT {ECO:0000250|UniProtKB:Q96E29}. FT CHAIN 68 412 Transcription termination factor 3, FT mitochondrial. FT /FTId=PRO_0000255458. FT CONFLICT 201 203 GPF -> DTY (in Ref. 1; BAB27006). FT {ECO:0000305}. SQ SEQUENCE 412 AA; 47357 MW; E85FAACAA83E1C59 CRC64; MALLAQQLPR RFNSVKLTSF IKAKQVTKRS ARAGKAVLPG FSAQPLLSSD TSFLRRGIKT YRTLFWNRFH SASTNRTKSS AESTLLPSVA EQQERILSLE SELPLEEVDD LPPLSPLQSV SEEEAIQIAA YSPLPISSFT LADYVDHSKT LQKLVQLGVD LSKIEKHPDA ANLLLRLDFE KHIKQILLFL KDLGLEDNQL GPFLTKNYAI FSEDLENLKT RVAYLQSKNF SKTDIARMVK NAPFLLSFSV ERLDNRLGFF QKELELNVKK TRDLVVRLPR LLTGSLEPVK ENMKVYHLEL GFKHNEIQHM VIKIPKMLTA NKRKLTEIFD YVHNVMNIPH HIIVKFPQLF NTRVFKIKER HLFLAYLGRA QYDPAKPNYV SLDKFVSFPD KIFCKEIAKA SLNDFEKFLK TL //