MITOK_MOUSE
ID MITOK_MOUSE Reviewed; 406 AA.
AC Q3URS9; Q4QQL1; Q9CXS1;
DT 29-MAY-2007, integrated into UniProtKB/Swiss-Prot.
DT 11-OCT-2005, sequence version 1.
DT 03-AUG-2022, entry version 104.
DE RecName: Full=Mitochondrial potassium channel {ECO:0000303|PubMed:31435016};
DE Short=MITOK {ECO:0000303|PubMed:31435016};
DE AltName: Full=Coiled-coil domain-containing protein 51;
DE Flags: Precursor;
GN Name=Ccdc51 {ECO:0000312|MGI:MGI:1913908};
GN Synonyms=Mitok {ECO:0000303|PubMed:31435016};
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] (ISOFORMS 1 AND 2).
RC STRAIN=C57BL/6J; TISSUE=Embryo, and Head;
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] (ISOFORM 2).
RC STRAIN=C57BL/6J; TISSUE=Brain, and Mammary gland;
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 IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
RC TISSUE=Brain, Brown adipose tissue, Heart, Kidney, Liver, and Spleen;
RX PubMed=21183079; DOI=10.1016/j.cell.2010.12.001;
RA Huttlin E.L., Jedrychowski M.P., Elias J.E., Goswami T., Rad R.,
RA Beausoleil S.A., Villen J., Haas W., Sowa M.E., Gygi S.P.;
RT "A tissue-specific atlas of mouse protein phosphorylation and expression.";
RL Cell 143:1174-1189(2010).
RN [4]
RP FUNCTION, SUBCELLULAR LOCATION, DISRUPTION PHENOTYPE, ACTIVITY REGULATION,
RP AND SUBUNIT.
RX PubMed=31435016; DOI=10.1038/s41586-019-1498-3;
RA Paggio A., Checchetto V., Campo A., Menabo R., Di Marco G., Di Lisa F.,
RA Szabo I., Rizzuto R., De Stefani D.;
RT "Identification of an ATP-sensitive potassium channel in mitochondria.";
RL Nature 572:609-613(2019).
CC -!- FUNCTION: Mitochondrial potassium channel located in the mitochondrial
CC inner membrane (PubMed:31435016). Together with ABCB8/MITOSUR, forms a
CC protein complex localized in the mitochondria that mediates ATP-
CC dependent potassium currents across the inner membrane (that is,
CC mitoK(ATP) channel) (PubMed:31435016). May contribute to the
CC homeostatic control of cellular metabolism under stress conditions by
CC regulating the mitochondrial matrix volume (PubMed:31435016).
CC {ECO:0000269|PubMed:31435016}.
CC -!- ACTIVITY REGULATION: Inhibited by ATP via mitoK(ATP) channel.
CC {ECO:0000269|PubMed:31435016}.
CC -!- SUBUNIT: The mitochondrial potassium channel (mitoK(ATP)) is composed
CC of 4 subunits of CCDC51/MITOK and 4 subunits of ABCB8/MITOSUR.
CC {ECO:0000305|PubMed:31435016}.
CC -!- SUBCELLULAR LOCATION: Mitochondrion inner membrane
CC {ECO:0000269|PubMed:31435016}; Multi-pass membrane protein
CC {ECO:0000255}.
CC -!- ALTERNATIVE PRODUCTS:
CC Event=Alternative splicing; Named isoforms=2;
CC Name=1;
CC IsoId=Q3URS9-1; Sequence=Displayed;
CC Name=2;
CC IsoId=Q3URS9-2; Sequence=VSP_025801;
CC -!- DISRUPTION PHENOTYPE: Knockout mice exhibit no visible phenotype
CC (PubMed:31435016). Mutant mice are slightly more sensitive to the
CC ischaemia-reperfusion protocol (PubMed:31435016).
CC {ECO:0000269|PubMed:31435016}.
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DR EMBL; AK014056; BAB29135.1; -; mRNA.
DR EMBL; AK137013; BAE23205.1; -; mRNA.
DR EMBL; AK141240; BAE24609.1; -; mRNA.
DR EMBL; BC098221; AAH98221.2; -; mRNA.
DR EMBL; BC116786; AAI16787.1; -; mRNA.
DR EMBL; BC116788; AAI16789.1; -; mRNA.
DR CCDS; CCDS52931.1; -. [Q3URS9-1]
DR RefSeq; NP_079965.2; NM_025689.4. [Q3URS9-1]
DR AlphaFoldDB; Q3URS9; -.
DR SMR; Q3URS9; -.
DR BioGRID; 211626; 2.
DR ComplexPortal; CPX-6084; MITOK-MITOSUR mitochondrial potassium channel complex.
DR IntAct; Q3URS9; 2.
DR STRING; 10090.ENSMUSP00000026735; -.
DR iPTMnet; Q3URS9; -.
DR PhosphoSitePlus; Q3URS9; -.
DR EPD; Q3URS9; -.
DR MaxQB; Q3URS9; -.
DR PaxDb; Q3URS9; -.
DR PeptideAtlas; Q3URS9; -.
DR PRIDE; Q3URS9; -.
DR ProteomicsDB; 281311; -. [Q3URS9-1]
DR ProteomicsDB; 281312; -. [Q3URS9-2]
DR Antibodypedia; 2566; 126 antibodies from 18 providers.
DR Ensembl; ENSMUST00000026735; ENSMUSP00000026735; ENSMUSG00000025645. [Q3URS9-1]
DR GeneID; 66658; -.
DR KEGG; mmu:66658; -.
DR UCSC; uc009rrx.2; mouse. [Q3URS9-1]
DR CTD; 79714; -.
DR MGI; MGI:1913908; Ccdc51.
DR VEuPathDB; HostDB:ENSMUSG00000025645; -.
DR eggNOG; ENOG502QWCS; Eukaryota.
DR GeneTree; ENSGT00390000001709; -.
DR HOGENOM; CLU_060968_0_0_1; -.
DR InParanoid; Q3URS9; -.
DR OMA; STATTWW; -.
DR OrthoDB; 1459041at2759; -.
DR PhylomeDB; Q3URS9; -.
DR TreeFam; TF318449; -.
DR BioGRID-ORCS; 66658; 1 hit in 71 CRISPR screens.
DR ChiTaRS; Ccdc51; mouse.
DR PRO; PR:Q3URS9; -.
DR Proteomes; UP000000589; Chromosome 9.
DR RNAct; Q3URS9; protein.
DR Bgee; ENSMUSG00000025645; Expressed in right kidney and 173 other tissues.
DR Genevisible; Q3URS9; MM.
DR GO; GO:0031305; C:integral component of mitochondrial inner membrane; IDA:UniProtKB.
DR GO; GO:0062157; C:mitochondrial ATP-gated potassium channel complex; IPI:ComplexPortal.
DR GO; GO:0005743; C:mitochondrial inner membrane; IDA:ComplexPortal.
DR GO; GO:0005739; C:mitochondrion; HDA:MGI.
DR GO; GO:0034705; C:potassium channel complex; IDA:UniProtKB.
DR GO; GO:0062156; F:mitochondrial ATP-gated potassium channel activity; IDA:UniProtKB.
DR GO; GO:0006884; P:cell volume homeostasis; ISO:MGI.
DR GO; GO:0140141; P:mitochondrial potassium ion transmembrane transport; ISO:MGI.
DR GO; GO:0071805; P:potassium ion transmembrane transport; IDA:UniProtKB.
DR InterPro; IPR037660; CCDC51.
DR PANTHER; PTHR28624; PTHR28624; 1.
PE 1: Evidence at protein level;
KW Alternative splicing; Coiled coil; Ion channel; Ion transport; Membrane;
KW Mitochondrion; Mitochondrion inner membrane; Potassium; Potassium channel;
KW Potassium transport; Reference proteome; Transit peptide; Transmembrane;
KW Transmembrane helix; Transport.
FT TRANSIT 1..35
FT /note="Mitochondrion"
FT /evidence="ECO:0000255"
FT CHAIN 36..406
FT /note="Mitochondrial potassium channel"
FT /evidence="ECO:0000255"
FT /id="PRO_0000288869"
FT TOPO_DOM 36..198
FT /note="Mitochondrial matrix"
FT /evidence="ECO:0000305|PubMed:31435016"
FT TRANSMEM 199..219
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TOPO_DOM 220..382
FT /note="Mitochondrial intermembrane"
FT /evidence="ECO:0000305|PubMed:31435016"
FT TRANSMEM 383..403
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TOPO_DOM 404..406
FT /note="Mitochondrial matrix"
FT /evidence="ECO:0000305|PubMed:31435016"
FT COILED 113..140
FT /evidence="ECO:0000255"
FT VAR_SEQ 1..31
FT /note="Missing (in isoform 2)"
FT /evidence="ECO:0000303|PubMed:15489334,
FT ECO:0000303|PubMed:16141072"
FT /id="VSP_025801"
FT CONFLICT 160
FT /note="R -> W (in Ref. 2; AAH98221)"
FT /evidence="ECO:0000305"
SQ SEQUENCE 406 AA; 45132 MW; D734FCFF6716354D CRC64;
MTGCSPVFAM QHVVGVPRIL VRRTFLGTDV TMTRTLCSPG PREKRPEAAA LGLFHRLPEL
GRTLSHTVRH QAASTAKAWW DRYEEFVGLN EVREAQGNVT EAEKVFMVAR GLVREAREGL
EAQQTKLKEV RDRLDRVSRE DNQYLELATL EHRMLQEEKR LRIAYLRAED SEREKFSLFS
AAVRESHEKE RTRAERTKNW SLIGSVLGAL IGVAGSTYVN RVRLQELKAL LLEAQKGPAS
LQEAIREQAS SYSLQQKDLQ DLMMDLRGLV HAEQGQGSGS PTGSSTRGKD IDGLSATMKE
QLRHSRQVYS CLEGLREQLD GLEKTCSQMA GVLQLAQAPA HPGTVGPVDG ALPSSLLEHG
SVILALSEME QRLEAQANRN TVSSTLVTCV TFLATLPLLY MLFKTS
