ID   CQ10B_HUMAN             Reviewed;         238 AA.
AC   Q9H8M1; B7Z1Y4;
DT   21-MAR-2006, integrated into UniProtKB/Swiss-Prot.
DT   01-MAR-2001, sequence version 1.
DT   03-AUG-2022, entry version 133.
DE   RecName: Full=Coenzyme Q-binding protein COQ10 homolog B, mitochondrial;
DE   Flags: Precursor;
GN   Name=COQ10B;
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 MRNA] (ISOFORMS 1 AND 2).
RC   TISSUE=Brain cortex, and Placenta;
RX   PubMed=14702039; DOI=10.1038/ng1285;
RA   Ota T., Suzuki Y., Nishikawa T., Otsuki T., Sugiyama T., Irie R.,
RA   Wakamatsu A., Hayashi K., Sato H., Nagai K., Kimura K., Makita H.,
RA   Sekine M., Obayashi M., Nishi T., Shibahara T., Tanaka T., Ishii S.,
RA   Yamamoto J., Saito K., Kawai Y., Isono Y., Nakamura Y., Nagahari K.,
RA   Murakami K., Yasuda T., Iwayanagi T., Wagatsuma M., Shiratori A., Sudo H.,
RA   Hosoiri T., Kaku Y., Kodaira H., Kondo H., Sugawara M., Takahashi M.,
RA   Kanda K., Yokoi T., Furuya T., Kikkawa E., Omura Y., Abe K., Kamihara K.,
RA   Katsuta N., Sato K., Tanikawa M., Yamazaki M., Ninomiya K., Ishibashi T.,
RA   Yamashita H., Murakawa K., Fujimori K., Tanai H., Kimata M., Watanabe M.,
RA   Hiraoka S., Chiba Y., Ishida S., Ono Y., Takiguchi S., Watanabe S.,
RA   Yosida M., Hotuta T., Kusano J., Kanehori K., Takahashi-Fujii A., Hara H.,
RA   Tanase T.-O., Nomura Y., Togiya S., Komai F., Hara R., Takeuchi K.,
RA   Arita M., Imose N., Musashino K., Yuuki H., Oshima A., Sasaki N.,
RA   Aotsuka S., Yoshikawa Y., Matsunawa H., Ichihara T., Shiohata N., Sano S.,
RA   Moriya S., Momiyama H., Satoh N., Takami S., Terashima Y., Suzuki O.,
RA   Nakagawa S., Senoh A., Mizoguchi H., Goto Y., Shimizu F., Wakebe H.,
RA   Hishigaki H., Watanabe T., Sugiyama A., Takemoto M., Kawakami B.,
RA   Yamazaki M., Watanabe K., Kumagai A., Itakura S., Fukuzumi Y., Fujimori Y.,
RA   Komiyama M., Tashiro H., Tanigami A., Fujiwara T., Ono T., Yamada K.,
RA   Fujii Y., Ozaki K., Hirao M., Ohmori Y., Kawabata A., Hikiji T.,
RA   Kobatake N., Inagaki H., Ikema Y., Okamoto S., Okitani R., Kawakami T.,
RA   Noguchi S., Itoh T., Shigeta K., Senba T., Matsumura K., Nakajima Y.,
RA   Mizuno T., Morinaga M., Sasaki M., Togashi T., Oyama M., Hata H.,
RA   Watanabe M., Komatsu T., Mizushima-Sugano J., Satoh T., Shirai Y.,
RA   Takahashi Y., Nakagawa K., Okumura K., Nagase T., Nomura N., Kikuchi H.,
RA   Masuho Y., Yamashita R., Nakai K., Yada T., Nakamura Y., Ohara O.,
RA   Isogai T., Sugano S.;
RT   "Complete sequencing and characterization of 21,243 full-length human
RT   cDNAs.";
RL   Nat. Genet. 36:40-45(2004).
RN   [2]
RP   NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
RX   PubMed=15815621; DOI=10.1038/nature03466;
RA   Hillier L.W., Graves T.A., Fulton R.S., Fulton L.A., Pepin K.H., Minx P.,
RA   Wagner-McPherson C., Layman D., Wylie K., Sekhon M., Becker M.C.,
RA   Fewell G.A., Delehaunty K.D., Miner T.L., Nash W.E., Kremitzki C., Oddy L.,
RA   Du H., Sun H., Bradshaw-Cordum H., Ali J., Carter J., Cordes M., Harris A.,
RA   Isak A., van Brunt A., Nguyen C., Du F., Courtney L., Kalicki J.,
RA   Ozersky P., Abbott S., Armstrong J., Belter E.A., Caruso L., Cedroni M.,
RA   Cotton M., Davidson T., Desai A., Elliott G., Erb T., Fronick C., Gaige T.,
RA   Haakenson W., Haglund K., Holmes A., Harkins R., Kim K., Kruchowski S.S.,
RA   Strong C.M., Grewal N., Goyea E., Hou S., Levy A., Martinka S., Mead K.,
RA   McLellan M.D., Meyer R., Randall-Maher J., Tomlinson C.,
RA   Dauphin-Kohlberg S., Kozlowicz-Reilly A., Shah N., Swearengen-Shahid S.,
RA   Snider J., Strong J.T., Thompson J., Yoakum M., Leonard S., Pearman C.,
RA   Trani L., Radionenko M., Waligorski J.E., Wang C., Rock S.M.,
RA   Tin-Wollam A.-M., Maupin R., Latreille P., Wendl M.C., Yang S.-P., Pohl C.,
RA   Wallis J.W., Spieth J., Bieri T.A., Berkowicz N., Nelson J.O., Osborne J.,
RA   Ding L., Meyer R., Sabo A., Shotland Y., Sinha P., Wohldmann P.E.,
RA   Cook L.L., Hickenbotham M.T., Eldred J., Williams D., Jones T.A., She X.,
RA   Ciccarelli F.D., Izaurralde E., Taylor J., Schmutz J., Myers R.M.,
RA   Cox D.R., Huang X., McPherson J.D., Mardis E.R., Clifton S.W., Warren W.C.,
RA   Chinwalla A.T., Eddy S.R., Marra M.A., Ovcharenko I., Furey T.S.,
RA   Miller W., Eichler E.E., Bork P., Suyama M., Torrents D., Waterston R.H.,
RA   Wilson R.K.;
RT   "Generation and annotation of the DNA sequences of human chromosomes 2 and
RT   4.";
RL   Nature 434:724-731(2005).
RN   [3]
RP   NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
RC   TISSUE=Brain;
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).
CC   -!- FUNCTION: Required for the function of coenzyme Q in the respiratory
CC       chain. May serve as a chaperone or may be involved in the transport of
CC       Q6 from its site of synthesis to the catalytic sites of the respiratory
CC       complexes (By similarity). {ECO:0000250}.
CC   -!- SUBUNIT: Interacts with coenzyme Q. {ECO:0000250}.
CC   -!- SUBCELLULAR LOCATION: Mitochondrion inner membrane {ECO:0000250};
CC       Peripheral membrane protein {ECO:0000250}; Matrix side {ECO:0000250}.
CC   -!- ALTERNATIVE PRODUCTS:
CC       Event=Alternative splicing; Named isoforms=2;
CC       Name=1;
CC         IsoId=Q9H8M1-1; Sequence=Displayed;
CC       Name=2;
CC         IsoId=Q9H8M1-2; Sequence=VSP_056949;
CC   -!- SIMILARITY: Belongs to the COQ10 family. {ECO:0000305}.
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DR   EMBL; AK023510; BAB14593.1; -; mRNA.
DR   EMBL; AK294093; BAH11670.1; -; mRNA.
DR   EMBL; AC010746; -; NOT_ANNOTATED_CDS; Genomic_DNA.
DR   EMBL; BC022453; AAH22453.1; -; mRNA.
DR   CCDS; CCDS2319.1; -. [Q9H8M1-1]
DR   RefSeq; NP_001307747.1; NM_001320818.1.
DR   RefSeq; NP_001307748.1; NM_001320819.1.
DR   RefSeq; NP_001307749.1; NM_001320820.1. [Q9H8M1-2]
DR   RefSeq; NP_079423.1; NM_025147.4. [Q9H8M1-1]
DR   AlphaFoldDB; Q9H8M1; -.
DR   SMR; Q9H8M1; -.
DR   BioGRID; 123186; 24.
DR   IntAct; Q9H8M1; 12.
DR   STRING; 9606.ENSP00000263960; -.
DR   GlyGen; Q9H8M1; 2 sites, 1 O-linked glycan (2 sites).
DR   iPTMnet; Q9H8M1; -.
DR   PhosphoSitePlus; Q9H8M1; -.
DR   BioMuta; COQ10B; -.
DR   DMDM; 74733854; -.
DR   EPD; Q9H8M1; -.
DR   jPOST; Q9H8M1; -.
DR   MassIVE; Q9H8M1; -.
DR   MaxQB; Q9H8M1; -.
DR   PaxDb; Q9H8M1; -.
DR   PeptideAtlas; Q9H8M1; -.
DR   PRIDE; Q9H8M1; -.
DR   ProteomicsDB; 6385; -.
DR   ProteomicsDB; 81217; -. [Q9H8M1-1]
DR   Antibodypedia; 56538; 37 antibodies from 12 providers.
DR   DNASU; 80219; -.
DR   Ensembl; ENST00000263960.7; ENSP00000263960.2; ENSG00000115520.9. [Q9H8M1-1]
DR   GeneID; 80219; -.
DR   KEGG; hsa:80219; -.
DR   MANE-Select; ENST00000263960.7; ENSP00000263960.2; NM_025147.5; NP_079423.1.
DR   UCSC; uc002uuh.2; human. [Q9H8M1-1]
DR   CTD; 80219; -.
DR   DisGeNET; 80219; -.
DR   GeneCards; COQ10B; -.
DR   HGNC; HGNC:25819; COQ10B.
DR   HPA; ENSG00000115520; Low tissue specificity.
DR   neXtProt; NX_Q9H8M1; -.
DR   OpenTargets; ENSG00000115520; -.
DR   PharmGKB; PA143485440; -.
DR   VEuPathDB; HostDB:ENSG00000115520; -.
DR   eggNOG; KOG3177; Eukaryota.
DR   GeneTree; ENSGT00940000155367; -.
DR   InParanoid; Q9H8M1; -.
DR   OMA; HEFIPYC; -.
DR   OrthoDB; 1517528at2759; -.
DR   PhylomeDB; Q9H8M1; -.
DR   TreeFam; TF314447; -.
DR   PathwayCommons; Q9H8M1; -.
DR   Reactome; R-HSA-611105; Respiratory electron transport.
DR   SignaLink; Q9H8M1; -.
DR   BioGRID-ORCS; 80219; 23 hits in 1076 CRISPR screens.
DR   ChiTaRS; COQ10B; human.
DR   GenomeRNAi; 80219; -.
DR   Pharos; Q9H8M1; Tdark.
DR   PRO; PR:Q9H8M1; -.
DR   Proteomes; UP000005640; Chromosome 2.
DR   RNAct; Q9H8M1; protein.
DR   Bgee; ENSG00000115520; Expressed in oocyte and 199 other tissues.
DR   ExpressionAtlas; Q9H8M1; baseline and differential.
DR   Genevisible; Q9H8M1; HS.
DR   GO; GO:0005743; C:mitochondrial inner membrane; IEA:UniProtKB-SubCell.
DR   GO; GO:0005739; C:mitochondrion; IBA:GO_Central.
DR   GO; GO:0048039; F:ubiquinone binding; IBA:GO_Central.
DR   GO; GO:0045333; P:cellular respiration; IBA:GO_Central.
DR   GO; GO:0006744; P:ubiquinone biosynthetic process; IBA:GO_Central.
DR   CDD; cd07813; COQ10p_like; 1.
DR   Gene3D; 3.30.530.20; -; 1.
DR   InterPro; IPR044996; COQ10-like.
DR   InterPro; IPR005031; COQ10_START.
DR   InterPro; IPR023393; START-like_dom_sf.
DR   PANTHER; PTHR12901; PTHR12901; 1.
DR   Pfam; PF03364; Polyketide_cyc; 1.
PE   2: Evidence at transcript level;
KW   Alternative splicing; Membrane; Mitochondrion;
KW   Mitochondrion inner membrane; Reference proteome; Transit peptide.
FT   TRANSIT         1..37
FT                   /note="Mitochondrion"
FT                   /evidence="ECO:0000255"
FT   CHAIN           38..238
FT                   /note="Coenzyme Q-binding protein COQ10 homolog B,
FT                   mitochondrial"
FT                   /id="PRO_0000228647"
FT   VAR_SEQ         1..43
FT                   /note="Missing (in isoform 2)"
FT                   /evidence="ECO:0000303|PubMed:14702039"
FT                   /id="VSP_056949"
FT   VARIANT         48
FT                   /note="L -> F (in dbSNP:rs34946819)"
FT                   /id="VAR_033823"
SQ   SEQUENCE   238 AA;  27071 MW;  078ECF9A377908D0 CRC64;
     MAARTGHTAL RRVVSGCRPK SATAAGAQAP VRNGRYLASC GILMSRTLPL HTSILPKEIC
     ARTFFKITAP LINKRKEYSE RRILGYSMQE MYDVVSGVED YKHFVPWCKK SDVISKRSGY
     CKTRLEIGFP PVLERYTSVV TLVKPHLVKA SCTDGRLFNH LETIWRFSPG LPGYPRTCTL
     DFSISFEFRS LLHSQLATLF FDEVVKQMVA AFERRACKLY GPETNIPREL MLHEVHHT