MIC10_HUMAN
ID MIC10_HUMAN Reviewed; 78 AA.
AC Q5TGZ0; Q96G68;
DT 03-OCT-2006, integrated into UniProtKB/Swiss-Prot.
DT 21-DEC-2004, sequence version 1.
DT 03-AUG-2022, entry version 127.
DE RecName: Full=MICOS complex subunit MIC10 {ECO:0000305};
DE AltName: Full=Mitochondrial inner membrane organizing system protein 1;
GN Name=MICOS10 {ECO:0000312|HGNC:HGNC:32068};
GN Synonyms=C1orf151, MIC10, MINOS1 {ECO:0000312|HGNC:HGNC:32068};
OS Homo sapiens (Human).
OC Eukaryota; Metazoa; Chordata; Craniata; Vertebrata; Euteleostomi; Mammalia;
OC Eutheria; Euarchontoglires; Primates; Haplorrhini; Catarrhini; Hominidae;
OC Homo.
OX NCBI_TaxID=9606;
RN [1]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
RX PubMed=16710414; DOI=10.1038/nature04727;
RA Gregory S.G., Barlow K.F., McLay K.E., Kaul R., Swarbreck D., Dunham A.,
RA Scott C.E., Howe K.L., Woodfine K., Spencer C.C.A., Jones M.C., Gillson C.,
RA Searle S., Zhou Y., Kokocinski F., McDonald L., Evans R., Phillips K.,
RA Atkinson A., Cooper R., Jones C., Hall R.E., Andrews T.D., Lloyd C.,
RA Ainscough R., Almeida J.P., Ambrose K.D., Anderson F., Andrew R.W.,
RA Ashwell R.I.S., Aubin K., Babbage A.K., Bagguley C.L., Bailey J.,
RA Beasley H., Bethel G., Bird C.P., Bray-Allen S., Brown J.Y., Brown A.J.,
RA Buckley D., Burton J., Bye J., Carder C., Chapman J.C., Clark S.Y.,
RA Clarke G., Clee C., Cobley V., Collier R.E., Corby N., Coville G.J.,
RA Davies J., Deadman R., Dunn M., Earthrowl M., Ellington A.G., Errington H.,
RA Frankish A., Frankland J., French L., Garner P., Garnett J., Gay L.,
RA Ghori M.R.J., Gibson R., Gilby L.M., Gillett W., Glithero R.J.,
RA Grafham D.V., Griffiths C., Griffiths-Jones S., Grocock R., Hammond S.,
RA Harrison E.S.I., Hart E., Haugen E., Heath P.D., Holmes S., Holt K.,
RA Howden P.J., Hunt A.R., Hunt S.E., Hunter G., Isherwood J., James R.,
RA Johnson C., Johnson D., Joy A., Kay M., Kershaw J.K., Kibukawa M.,
RA Kimberley A.M., King A., Knights A.J., Lad H., Laird G., Lawlor S.,
RA Leongamornlert D.A., Lloyd D.M., Loveland J., Lovell J., Lush M.J.,
RA Lyne R., Martin S., Mashreghi-Mohammadi M., Matthews L., Matthews N.S.W.,
RA McLaren S., Milne S., Mistry S., Moore M.J.F., Nickerson T., O'Dell C.N.,
RA Oliver K., Palmeiri A., Palmer S.A., Parker A., Patel D., Pearce A.V.,
RA Peck A.I., Pelan S., Phelps K., Phillimore B.J., Plumb R., Rajan J.,
RA Raymond C., Rouse G., Saenphimmachak C., Sehra H.K., Sheridan E.,
RA Shownkeen R., Sims S., Skuce C.D., Smith M., Steward C., Subramanian S.,
RA Sycamore N., Tracey A., Tromans A., Van Helmond Z., Wall M., Wallis J.M.,
RA White S., Whitehead S.L., Wilkinson J.E., Willey D.L., Williams H.,
RA Wilming L., Wray P.W., Wu Z., Coulson A., Vaudin M., Sulston J.E.,
RA Durbin R.M., Hubbard T., Wooster R., Dunham I., Carter N.P., McVean G.,
RA Ross M.T., Harrow J., Olson M.V., Beck S., Rogers J., Bentley D.R.;
RT "The DNA sequence and biological annotation of human chromosome 1.";
RL Nature 441:315-321(2006).
RN [2]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 2).
RC TISSUE=Uterus;
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 IN THE MICOS COMPLEX, FUNCTION, SUBCELLULAR LOCATION, AND
RP TOPOLOGY.
RX PubMed=22114354; DOI=10.1091/mbc.e11-09-0774;
RA Alkhaja A.K., Jans D.C., Nikolov M., Vukotic M., Lytovchenko O.,
RA Ludewig F., Schliebs W., Riedel D., Urlaub H., Jakobs S., Deckers M.;
RT "MINOS1 is a conserved component of mitofilin complexes and required for
RT mitochondrial function and cristae organization.";
RL Mol. Biol. Cell 23:247-257(2012).
RN [4]
RP ACETYLATION [LARGE SCALE ANALYSIS] AT SER-2, CLEAVAGE OF INITIATOR
RP METHIONINE [LARGE SCALE ANALYSIS], AND IDENTIFICATION BY MASS SPECTROMETRY
RP [LARGE SCALE ANALYSIS].
RX PubMed=22814378; DOI=10.1073/pnas.1210303109;
RA Van Damme P., Lasa M., Polevoda B., Gazquez C., Elosegui-Artola A.,
RA Kim D.S., De Juan-Pardo E., Demeyer K., Hole K., Larrea E., Timmerman E.,
RA Prieto J., Arnesen T., Sherman F., Gevaert K., Aldabe R.;
RT "N-terminal acetylome analyses and functional insights of the N-terminal
RT acetyltransferase NatB.";
RL Proc. Natl. Acad. Sci. U.S.A. 109:12449-12454(2012).
RN [5]
RP NOMENCLATURE.
RX PubMed=24687277; DOI=10.1083/jcb.201401006;
RA Pfanner N., van der Laan M., Amati P., Capaldi R.A., Caudy A.A.,
RA Chacinska A., Darshi M., Deckers M., Hoppins S., Icho T., Jakobs S., Ji J.,
RA Kozjak-Pavlovic V., Meisinger C., Odgren P.R., Park S.K., Rehling P.,
RA Reichert A.S., Sheikh M.S., Taylor S.S., Tsuchida N., van der Bliek A.M.,
RA van der Klei I.J., Weissman J.S., Westermann B., Zha J., Neupert W.,
RA Nunnari J.;
RT "Uniform nomenclature for the mitochondrial contact site and cristae
RT organizing system.";
RL J. Cell Biol. 204:1083-1086(2014).
RN [6]
RP INTERACTION WITH APOO; APOOL AND IMMT.
RX PubMed=25764979; DOI=10.1016/j.bbamcr.2015.03.004;
RA Koob S., Barrera M., Anand R., Reichert A.S.;
RT "The non-glycosylated isoform of MIC26 is a constituent of the mammalian
RT MICOS complex and promotes formation of crista junctions.";
RL Biochim. Biophys. Acta 1853:1551-1563(2015).
RN [7]
RP INTERACTION WITH MICOS13 AND IMMT.
RX PubMed=25997101; DOI=10.7554/elife.06265;
RA Guarani V., McNeill E.M., Paulo J.A., Huttlin E.L., Froehlich F.,
RA Gygi S.P., Van Vactor D., Harper J.W.;
RT "QIL1 is a novel mitochondrial protein required for MICOS complex stability
RT and cristae morphology.";
RL Elife 4:0-0(2015).
RN [8]
RP ACETYLATION [LARGE SCALE ANALYSIS] AT SER-2, CLEAVAGE OF INITIATOR
RP METHIONINE [LARGE SCALE ANALYSIS], AND IDENTIFICATION BY MASS SPECTROMETRY
RP [LARGE SCALE ANALYSIS].
RX PubMed=25944712; DOI=10.1002/pmic.201400617;
RA Vaca Jacome A.S., Rabilloud T., Schaeffer-Reiss C., Rompais M., Ayoub D.,
RA Lane L., Bairoch A., Van Dorsselaer A., Carapito C.;
RT "N-terminome analysis of the human mitochondrial proteome.";
RL Proteomics 15:2519-2524(2015).
RN [9]
RP INTERACTION WITH ARMC1.
RX PubMed=31644573; DOI=10.1371/journal.pone.0218303;
RA Wagner F., Kunz T.C., Chowdhury S.R., Thiede B., Fraunholz M., Eger D.,
RA Kozjak-Pavlovic V.;
RT "Armadillo repeat-containing protein 1 is a dual localization protein
RT associated with mitochondrial intermembrane space bridging complex.";
RL PLoS ONE 14:e0218303-e0218303(2019).
CC -!- FUNCTION: Component of the MICOS complex, a large protein complex of
CC the mitochondrial inner membrane that plays crucial roles in the
CC maintenance of crista junctions, inner membrane architecture, and
CC formation of contact sites to the outer membrane.
CC {ECO:0000269|PubMed:22114354}.
CC -!- SUBUNIT: Component of the mitochondrial contact site and cristae
CC organizing system (MICOS) complex, composed of at least MICOS10/MIC10,
CC CHCHD3/MIC19, CHCHD6/MIC25, APOOL/MIC27, IMMT/MIC60, APOO/MIC23/MIC26
CC and MICOS13/MIC13. This complex was also known under the names MINOS or
CC MitOS complex. The MICOS complex associates with mitochondrial outer
CC membrane proteins SAMM50, MTX1 and MTX2 (together described as
CC components of the mitochondrial outer membrane sorting assembly
CC machinery (SAM) complex) and DNAJC11, mitochondrial inner membrane
CC protein TMEM11 and with HSPA9. The MICOS and SAM complexes together
CC with DNAJC11 are part of a large protein complex spanning both
CC membranes termed the mitochondrial intermembrane space bridging (MIB)
CC complex. Interacts with IMMT/MIC60 and MICOS13/MIC13. Interacts with
CC APOO/MIC23/MIC26 and APOOL/MIC27. Interacts with ARMC1
CC (PubMed:31644573). {ECO:0000269|PubMed:22114354,
CC ECO:0000269|PubMed:25764979, ECO:0000269|PubMed:25997101,
CC ECO:0000269|PubMed:31644573}.
CC -!- INTERACTION:
CC Q5TGZ0; P55056: APOC4; NbExp=3; IntAct=EBI-12886442, EBI-18302142;
CC Q5TGZ0; Q9UHD4: CIDEB; NbExp=3; IntAct=EBI-12886442, EBI-7062247;
CC Q5TGZ0; O95563: MPC2; NbExp=3; IntAct=EBI-12886442, EBI-719403;
CC -!- SUBCELLULAR LOCATION: Mitochondrion inner membrane
CC {ECO:0000269|PubMed:22114354}; Single-pass membrane protein
CC {ECO:0000269|PubMed:22114354}. Note=The C-terminus is located in the
CC intermembrane space (By similarity), while the location of the N-
CC terminus has not been determined yet. As some programs predict the
CC presence of 2 closely apposed membrane domains, it has been proposed
CC that the protein may cross the membrane twice and that both termini may
CC face the intermembrane space (PubMed:22114354). {ECO:0000250,
CC ECO:0000269|PubMed:22114354}.
CC -!- ALTERNATIVE PRODUCTS:
CC Event=Alternative splicing; Named isoforms=2;
CC Name=1;
CC IsoId=Q5TGZ0-1; Sequence=Displayed;
CC Name=2;
CC IsoId=Q5TGZ0-2; Sequence=VSP_042062, VSP_042063;
CC -!- SIMILARITY: Belongs to the MICOS complex subunit Mic10 family.
CC {ECO:0000305}.
CC -!- SEQUENCE CAUTION:
CC Sequence=AAH09927.1; Type=Erroneous initiation; Note=Extended N-terminus.; Evidence={ECO:0000305};
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DR EMBL; AL031727; -; NOT_ANNOTATED_CDS; Genomic_DNA.
DR EMBL; BC009927; AAH09927.1; ALT_INIT; mRNA.
DR EMBL; BC070388; AAH70388.1; -; mRNA.
DR CCDS; CCDS30620.1; -. [Q5TGZ0-1]
DR RefSeq; NP_001027535.1; NM_001032363.3. [Q5TGZ0-1]
DR AlphaFoldDB; Q5TGZ0; -.
DR BioGRID; 136699; 83.
DR ComplexPortal; CPX-6141; MICOS mitochondrial contact site and cristae organizing system complex.
DR CORUM; Q5TGZ0; -.
DR IntAct; Q5TGZ0; 3.
DR STRING; 9606.ENSP00000325562; -.
DR ChEMBL; CHEMBL4105956; -.
DR TCDB; 8.A.156.1.2; the micos complex (micos-c) family.
DR iPTMnet; Q5TGZ0; -.
DR PhosphoSitePlus; Q5TGZ0; -.
DR BioMuta; MINOS1; -.
DR DMDM; 74746535; -.
DR EPD; Q5TGZ0; -.
DR jPOST; Q5TGZ0; -.
DR MassIVE; Q5TGZ0; -.
DR MaxQB; Q5TGZ0; -.
DR PaxDb; Q5TGZ0; -.
DR PeptideAtlas; Q5TGZ0; -.
DR PRIDE; Q5TGZ0; -.
DR ProteomicsDB; 65135; -. [Q5TGZ0-1]
DR ProteomicsDB; 65136; -. [Q5TGZ0-2]
DR TopDownProteomics; Q5TGZ0-1; -. [Q5TGZ0-1]
DR Antibodypedia; 29686; 101 antibodies from 18 providers.
DR DNASU; 440574; -.
DR Ensembl; ENST00000322753.7; ENSP00000325562.6; ENSG00000173436.15. [Q5TGZ0-1]
DR GeneID; 440574; -.
DR KEGG; hsa:440574; -.
DR MANE-Select; ENST00000322753.7; ENSP00000325562.6; NM_001032363.4; NP_001027535.1.
DR UCSC; uc001bci.3; human. [Q5TGZ0-1]
DR CTD; 440574; -.
DR DisGeNET; 440574; -.
DR GeneCards; MICOS10; -.
DR HGNC; HGNC:32068; MICOS10.
DR HPA; ENSG00000173436; Low tissue specificity.
DR MIM; 616574; gene.
DR neXtProt; NX_Q5TGZ0; -.
DR PharmGKB; PA142672468; -.
DR VEuPathDB; HostDB:ENSG00000173436; -.
DR eggNOG; KOG4604; Eukaryota.
DR GeneTree; ENSGT00390000005732; -.
DR HOGENOM; CLU_068905_2_1_1; -.
DR InParanoid; Q5TGZ0; -.
DR OMA; NCRHDFA; -.
DR PhylomeDB; Q5TGZ0; -.
DR TreeFam; TF300259; -.
DR PathwayCommons; Q5TGZ0; -.
DR Reactome; R-HSA-8949613; Cristae formation.
DR SignaLink; Q5TGZ0; -.
DR BioGRID-ORCS; 440574; 338 hits in 1026 CRISPR screens.
DR ChiTaRS; MINOS1; human.
DR GenomeRNAi; 440574; -.
DR Pharos; Q5TGZ0; Tdark.
DR PRO; PR:Q5TGZ0; -.
DR Proteomes; UP000005640; Chromosome 1.
DR RNAct; Q5TGZ0; protein.
DR Bgee; ENSG00000173436; Expressed in mucosa of transverse colon and 144 other tissues.
DR ExpressionAtlas; Q5TGZ0; baseline and differential.
DR Genevisible; Q5TGZ0; HS.
DR GO; GO:0140275; C:MIB complex; HDA:UniProtKB.
DR GO; GO:0061617; C:MICOS complex; HDA:UniProtKB.
DR GO; GO:0044284; C:mitochondrial crista junction; IC:ComplexPortal.
DR GO; GO:0005743; C:mitochondrial inner membrane; IC:ComplexPortal.
DR GO; GO:0005739; C:mitochondrion; IDA:HPA.
DR GO; GO:0001401; C:SAM complex; HDA:UniProtKB.
DR GO; GO:0042407; P:cristae formation; IC:ComplexPortal.
DR GO; GO:0007007; P:inner mitochondrial membrane organization; IC:UniProtKB.
DR InterPro; IPR007512; Mic10.
DR PANTHER; PTHR21304; PTHR21304; 1.
DR Pfam; PF04418; DUF543; 1.
PE 1: Evidence at protein level;
KW Acetylation; Alternative splicing; Membrane; Mitochondrion;
KW Mitochondrion inner membrane; Reference proteome; Transmembrane;
KW Transmembrane helix.
FT INIT_MET 1
FT /note="Removed"
FT /evidence="ECO:0007744|PubMed:22814378,
FT ECO:0007744|PubMed:25944712"
FT CHAIN 2..78
FT /note="MICOS complex subunit MIC10"
FT /id="PRO_0000249459"
FT TRANSMEM 17..36
FT /note="Helical"
FT /evidence="ECO:0000255"
FT TOPO_DOM 37..78
FT /note="Mitochondrial intermembrane"
FT /evidence="ECO:0000255"
FT MOD_RES 2
FT /note="N-acetylserine"
FT /evidence="ECO:0007744|PubMed:22814378,
FT ECO:0007744|PubMed:25944712"
FT VAR_SEQ 22..24
FT /note="GTG -> ESW (in isoform 2)"
FT /evidence="ECO:0000303|PubMed:15489334"
FT /id="VSP_042062"
FT VAR_SEQ 25..78
FT /note="Missing (in isoform 2)"
FT /evidence="ECO:0000303|PubMed:15489334"
FT /id="VSP_042063"
SQ SEQUENCE 78 AA; 8808 MW; 6E4F618F07E969BE CRC64;
MSESELGRKW DRCLADAVVK IGTGFGLGIV FSLTFFKRRM WPLAFGSGMG LGMAYSNCQH
DFQAPYLLHG KYVKEQEQ
