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 24-JUL-2024, entry version 126. 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; Mammalia; OC Eutheria; Euarchontoglires; Glires; Rodentia; Myomorpha; Muroidea; Muridae; OC 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., Davis M.J., RA Wilming L.G., Aidinis V., Allen J.E., Ambesi-Impiombato A., Apweiler R., RA Aturaliya R.N., Bailey T.L., Bansal M., Baxter L., Beisel K.W., Bersano T., RA Bono H., Chalk A.M., Chiu K.P., Choudhary V., Christoffels A., RA Clutterbuck D.R., Crowe M.L., Dalla E., Dalrymple B.P., de Bono B., RA Della Gatta G., di Bernardo D., Down T., Engstrom P., Fagiolini M., RA Faulkner G., Fletcher C.F., Fukushima T., Furuno M., Futaki S., RA Gariboldi M., Georgii-Hemming P., Gingeras T.R., Gojobori T., Green R.E., RA Gustincich S., Harbers M., Hayashi Y., Hensch T.K., Hirokawa N., Hill D., RA Huminiecki L., Iacono M., Ikeo K., Iwama A., Ishikawa T., Jakt M., RA Kanapin A., Katoh M., Kawasawa Y., Kelso J., Kitamura H., Kitano H., RA Kollias G., Krishnan S.P., Kruger A., Kummerfeld S.K., Kurochkin I.V., RA Lareau L.F., Lazarevic D., Lipovich L., Liu J., Liuni S., McWilliam S., RA Madan Babu M., Madera M., Marchionni L., Matsuda H., Matsuzawa S., Miki H., RA Mignone F., Miyake S., Morris K., Mottagui-Tabar S., Mulder N., Nakano N., RA Nakauchi H., Ng P., Nilsson R., Nishiguchi S., Nishikawa S., Nori F., RA Ohara O., Okazaki Y., Orlando V., Pang K.C., Pavan W.J., Pavesi G., RA Pesole G., Petrovsky N., Piazza S., Reed J., Reid J.F., Ring B.Z., RA Ringwald M., 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., Sperling S., RA Stupka E., Sugiura K., Sultana R., Takenaka Y., Taki K., Tammoja K., RA Tan S.L., Tang S., Taylor M.S., Tegner J., Teichmann S.A., Ueda H.R., RA van Nimwegen E., Verardo R., Wei C.L., Yagi K., Yamanishi H., RA Zabarovsky E., Zhu S., Zimmer A., Hide W., Bult C., Grimmond S.M., RA Teasdale R.D., Liu E.T., Brusic V., Quackenbush J., Wahlestedt C., RA Mattick J.S., Hume D.A., Kai C., Sasaki D., Tomaru Y., Fukuda S., RA Kanamori-Katayama M., Suzuki M., Aoki J., Arakawa T., Iida J., Imamura K., RA Itoh M., Kato T., Kawaji H., Kawagashira N., Kawashima T., Kojima M., RA Kondo S., Konno H., Nakano K., Ninomiya N., Nishio T., Okada M., Plessy C., RA Shibata K., Shiraki T., Suzuki S., Tagami M., Waki K., Watahiki A., RA Okamura-Oho Y., Suzuki H., Kawai J., 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 project: RT 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., Gaspari M., RA Wibom R., Hultenby K., Erdjument-Bromage H., Tempst P., Falkenberg M., RA 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., Mourier A., RA Freyer C., Ruzzenente B., Tain L., Groenke S., Baggio F., Kukat C., RA Kremmer E., Wibom R., Polosa P.L., Habermann B., Partridge L., Park C.B., RA Larsson N.G.; RT "MTERF3 regulates mitochondrial ribosome biogenesis in invertebrates and RT mammals."; RL PLoS Genet. 9:E1003178-E1003178(2013). CC -!- FUNCTION: Binds promoter DNA and regulates initiation of transcription CC (By similarity). Required for normal mitochondrial transcription and CC translation, and for normal assembly of mitochondrial respiratory CC complexes (PubMed:17662942, PubMed:23300484). Required for normal CC mitochondrial function (PubMed:17662942, PubMed:23300484). Maintains CC 16S rRNA levels and functions in mitochondrial ribosome assembly by CC regulating the biogenesis of the 39S ribosomal subunit CC (PubMed:17662942, PubMed:23300484). {ECO:0000250|UniProtKB:Q96E29, CC ECO:0000269|PubMed:17662942, ECO:0000269|PubMed:23300484}. CC -!- SUBCELLULAR LOCATION: Mitochondrion {ECO:0000250|UniProtKB:Q96E29}. CC -!- DOMAIN: Contains seven structural repeats of about 35 residues, where CC each repeat contains three helices. The repeats form a superhelical CC structure with a solenoid shape. {ECO:0000250|UniProtKB:Q96E29}. CC -!- DISRUPTION PHENOTYPE: Embryonic lethality, due to severe mitochondrial CC dysfunction. Embryos are much smaller than normal and none survive past CC 10.5 dpc. {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 AlphaFoldDB; Q8R3J4; -. DR SMR; Q8R3J4; -. DR STRING; 10090.ENSMUSP00000021991; -. DR iPTMnet; Q8R3J4; -. DR PhosphoSitePlus; Q8R3J4; -. DR PaxDb; 10090-ENSMUSP00000021991; -. DR PeptideAtlas; Q8R3J4; -. DR ProteomicsDB; 286072; -. DR Pumba; Q8R3J4; -. DR Antibodypedia; 1268; 63 antibodies from 22 providers. DR DNASU; 66410; -. DR Ensembl; ENSMUST00000021991.11; ENSMUSP00000021991.5; ENSMUSG00000021519.12. DR GeneID; 66410; -. DR KEGG; mmu:66410; -. DR UCSC; uc007qzw.2; mouse. DR AGR; MGI:1913660; -. DR CTD; 51001; -. DR MGI; MGI:1913660; Mterf3. DR VEuPathDB; HostDB:ENSMUSG00000021519; -. DR eggNOG; KOG1267; Eukaryota. DR GeneTree; ENSGT00390000005801; -. DR HOGENOM; CLU_042536_0_0_1; -. DR InParanoid; Q8R3J4; -. DR OMA; EPRIIMF; -. DR OrthoDB; 2915809at2759; -. DR PhylomeDB; Q8R3J4; -. DR TreeFam; TF317943; -. DR Reactome; R-MMU-5205685; PINK1-PRKN Mediated Mitophagy. DR BioGRID-ORCS; 66410; 18 hits in 77 CRISPR screens. DR ChiTaRS; Mterf3; mouse. DR PRO; PR:Q8R3J4; -. DR Proteomes; UP000000589; Chromosome 13. DR RNAct; Q8R3J4; Protein. DR Bgee; ENSMUSG00000021519; Expressed in medial ganglionic eminence and 263 other cell types or tissues. DR ExpressionAtlas; Q8R3J4; baseline and differential. DR GO; GO:0005829; C:cytosol; IEA:Ensembl. DR GO; GO:0005739; C:mitochondrion; HDA:MGI. DR GO; GO:0000976; F:transcription cis-regulatory region 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:0045892; P:negative regulation of DNA-templated transcription; IMP:UniProtKB. DR Gene3D; 1.25.70.10; Transcription termination factor 3, mitochondrial; 1. DR InterPro; IPR003690; MTERF. DR InterPro; IPR038538; MTERF_sf. DR PANTHER; PTHR13068; CGI-12 PROTEIN-RELATED; 1. DR PANTHER; PTHR13068:SF194; TRANSCRIPTION TERMINATION FACTOR 3, MITOCHONDRIAL; 1. DR Pfam; PF02536; mTERF; 1. DR SMART; SM00733; Mterf; 6. PE 2: Evidence at transcript level; KW DNA-binding; Mitochondrion; Reference proteome; Ribosome biogenesis; KW Transcription; Transcription regulation; Transit peptide. FT TRANSIT 1..67 FT /note="Mitochondrion" FT /evidence="ECO:0000250|UniProtKB:Q96E29" FT CHAIN 68..412 FT /note="Transcription termination factor 3, mitochondrial" FT /id="PRO_0000255458" FT CONFLICT 201..203 FT /note="GPF -> DTY (in Ref. 1; BAB27006)" FT /evidence="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 //