UQCC1_MOUSE
ID UQCC1_MOUSE Reviewed; 295 AA.
AC Q9CWU6; A2ARK1; Q3TET9; Q9CRP2; Q9JK78;
DT 22-FEB-2003, integrated into UniProtKB/Swiss-Prot.
DT 01-JUN-2001, sequence version 1.
DT 03-AUG-2022, entry version 139.
DE RecName: Full=Ubiquinol-cytochrome-c reductase complex assembly factor 1;
DE AltName: Full=Basic FGF-repressed Zic-binding protein;
DE Short=mbFZb;
DE AltName: Full=Ubiquinol-cytochrome c reductase complex chaperone CBP3 homolog;
GN Name=Uqcc1; Synonyms=Bfzb, Uqcc;
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;
RC TISSUE=Egg, Embryo, Embryonic head, Embryonic stem cell, and Pituitary;
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 GENOMIC DNA].
RC STRAIN=C57BL/6J;
RX PubMed=19468303; DOI=10.1371/journal.pbio.1000112;
RA Church D.M., Goodstadt L., Hillier L.W., Zody M.C., Goldstein S., She X.,
RA Bult C.J., Agarwala R., Cherry J.L., DiCuccio M., Hlavina W., Kapustin Y.,
RA Meric P., Maglott D., Birtle Z., Marques A.C., Graves T., Zhou S.,
RA Teague B., Potamousis K., Churas C., Place M., Herschleb J., Runnheim R.,
RA Forrest D., Amos-Landgraf J., Schwartz D.C., Cheng Z., Lindblad-Toh K.,
RA Eichler E.E., Ponting C.P.;
RT "Lineage-specific biology revealed by a finished genome assembly of the
RT mouse.";
RL PLoS Biol. 7:E1000112-E1000112(2009).
RN [3]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORM 1).
RC STRAIN=C57BL/6J; 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).
RN [4]
RP NUCLEOTIDE SEQUENCE [MRNA] OF 3-295 (ISOFORM 1), TISSUE SPECIFICITY,
RP DEVELOPMENTAL STAGE, AND INDUCTION.
RC TISSUE=Embryonic stem cell;
RX PubMed=11118897; DOI=10.1016/s0925-4773(00)00511-6;
RA Vetter K., Wurst W.;
RT "Expression of a novel mouse gene 'mbFZb' in distinct regions of the
RT developing nervous system and the adult brain.";
RL Mech. Dev. 100:123-125(2001).
RN [5]
RP IDENTIFICATION BY MASS SPECTROMETRY [LARGE SCALE ANALYSIS].
RC TISSUE=Brain, Brown adipose tissue, Heart, Kidney, Liver, Lung, and Testis;
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).
CC -!- FUNCTION: Required for the assembly of the ubiquinol-cytochrome c
CC reductase complex (mitochondrial respiratory chain complex III or
CC cytochrome b-c1 complex). Involved in cytochrome b translation and/or
CC stability. {ECO:0000250|UniProtKB:Q9NVA1}.
CC -!- SUBUNIT: Interacts with UQCC2. {ECO:0000250|UniProtKB:Q9NVA1}.
CC -!- SUBCELLULAR LOCATION: Mitochondrion inner membrane
CC {ECO:0000250|UniProtKB:Q9NVA1}. Cytoplasmic vesicle {ECO:0000305}.
CC Note=Cytoplasmic vesicular structures. {ECO:0000305}.
CC -!- ALTERNATIVE PRODUCTS:
CC Event=Alternative splicing; Named isoforms=2;
CC Name=1;
CC IsoId=Q9CWU6-1; Sequence=Displayed;
CC Name=2;
CC IsoId=Q9CWU6-2; Sequence=VSP_000858, VSP_000859;
CC -!- TISSUE SPECIFICITY: In the brain it is restricted to the olfactory
CC bulb, the hippocampus, the piriform cortex and the Purkinje cells.
CC {ECO:0000269|PubMed:11118897}.
CC -!- DEVELOPMENTAL STAGE: Expressed mainly in the developing nervous system
CC from 9.5 dpc onwards. Also detected in the developing eye and the brown
CC fat. {ECO:0000269|PubMed:11118897}.
CC -!- INDUCTION: Repressed by bFGF; in embryonic stem cells.
CC {ECO:0000269|PubMed:11118897}.
CC -!- SIMILARITY: Belongs to the CBP3 family. {ECO:0000305}.
CC -!- SEQUENCE CAUTION:
CC Sequence=AAF64519.1; Type=Erroneous initiation; Note=Truncated N-terminus.; Evidence={ECO:0000305};
CC Sequence=BAB27433.1; Type=Erroneous termination; Note=Truncated C-terminus.; Evidence={ECO:0000305};
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DR EMBL; AK010376; BAB26894.1; -; mRNA.
DR EMBL; AK011152; BAB27433.1; ALT_SEQ; mRNA.
DR EMBL; AK019425; -; NOT_ANNOTATED_CDS; mRNA.
DR EMBL; AK077323; BAC36749.1; -; mRNA.
DR EMBL; AK139488; BAE24034.1; -; mRNA.
DR EMBL; AK169413; BAE41159.1; -; mRNA.
DR EMBL; AL845445; -; NOT_ANNOTATED_CDS; Genomic_DNA.
DR EMBL; BC050884; AAH50884.1; -; mRNA.
DR EMBL; BC057570; AAH57570.1; -; mRNA.
DR EMBL; AF253516; AAF64519.1; ALT_INIT; mRNA.
DR CCDS; CCDS16957.2; -. [Q9CWU6-1]
DR RefSeq; NP_061376.2; NM_018888.4. [Q9CWU6-1]
DR AlphaFoldDB; Q9CWU6; -.
DR SMR; Q9CWU6; -.
DR BioGRID; 207792; 2.
DR STRING; 10090.ENSMUSP00000105264; -.
DR iPTMnet; Q9CWU6; -.
DR PhosphoSitePlus; Q9CWU6; -.
DR SwissPalm; Q9CWU6; -.
DR EPD; Q9CWU6; -.
DR MaxQB; Q9CWU6; -.
DR PaxDb; Q9CWU6; -.
DR PeptideAtlas; Q9CWU6; -.
DR PRIDE; Q9CWU6; -.
DR ProteomicsDB; 299643; -. [Q9CWU6-1]
DR ProteomicsDB; 299644; -. [Q9CWU6-2]
DR Antibodypedia; 26044; 35 antibodies from 12 providers.
DR DNASU; 56046; -.
DR Ensembl; ENSMUST00000109636; ENSMUSP00000105264; ENSMUSG00000005882. [Q9CWU6-1]
DR GeneID; 56046; -.
DR KEGG; mmu:56046; -.
DR UCSC; uc008nlo.2; mouse. [Q9CWU6-1]
DR CTD; 55245; -.
DR MGI; MGI:1929472; Uqcc1.
DR VEuPathDB; HostDB:ENSMUSG00000005882; -.
DR eggNOG; KOG2873; Eukaryota.
DR GeneTree; ENSGT00390000018118; -.
DR InParanoid; Q9CWU6; -.
DR OMA; QLYYNCA; -.
DR OrthoDB; 1428265at2759; -.
DR PhylomeDB; Q9CWU6; -.
DR TreeFam; TF313220; -.
DR BioGRID-ORCS; 56046; 16 hits in 73 CRISPR screens.
DR ChiTaRS; Uqcc1; mouse.
DR PRO; PR:Q9CWU6; -.
DR Proteomes; UP000000589; Chromosome 2.
DR RNAct; Q9CWU6; protein.
DR Bgee; ENSMUSG00000005882; Expressed in hindlimb stylopod muscle and 273 other tissues.
DR ExpressionAtlas; Q9CWU6; baseline and differential.
DR Genevisible; Q9CWU6; MM.
DR GO; GO:0031410; C:cytoplasmic vesicle; IDA:MGI.
DR GO; GO:0005743; C:mitochondrial inner membrane; ISS:UniProtKB.
DR GO; GO:0005739; C:mitochondrion; HDA:MGI.
DR GO; GO:0034551; P:mitochondrial respiratory chain complex III assembly; ISS:UniProtKB.
DR InterPro; IPR021150; Ubiq_cyt_c_chap.
DR InterPro; IPR007129; Ubiqinol_cyt_c_chaperone_CPB3.
DR PANTHER; PTHR12184; PTHR12184; 1.
DR Pfam; PF03981; Ubiq_cyt_C_chap; 1.
PE 1: Evidence at protein level;
KW Alternative splicing; Cytoplasmic vesicle; Membrane; Mitochondrion;
KW Mitochondrion inner membrane; Reference proteome.
FT CHAIN 1..295
FT /note="Ubiquinol-cytochrome-c reductase complex assembly
FT factor 1"
FT /id="PRO_0000206561"
FT VAR_SEQ 70
FT /note="S -> T (in isoform 2)"
FT /evidence="ECO:0000305"
FT /id="VSP_000858"
FT VAR_SEQ 71..207
FT /note="Missing (in isoform 2)"
FT /evidence="ECO:0000305"
FT /id="VSP_000859"
FT CONFLICT 97
FT /note="G -> V (in Ref. 1; BAC36749)"
FT /evidence="ECO:0000305"
SQ SEQUENCE 295 AA; 34300 MW; AB7D0C9BF6F7B00A CRC64;
MALLVRVLRN QTSISQWVPV CSQLVSVSPT QRQWSSTSQW LQKNQSRVCL GSEQTVGADT
AQNRKYHNTS KLLTTQDFPQ PVEEKVGPFT KIIEAMGFTG PLKYSKWKIK IAALRMYTSC
VEKTDFEEFF LRCQMPDTFN SWFLITLLHV WMCLVRMKQE GRTGKYMCRI IVHFMWEDVE
QRGRVMGVNS YILKKNMALM TNNFYAAILG YDEGILSDDH GLAAALWRTF FNQKCEDPRQ
LELLVEYVRK QMQYLDSMNG EDLLLTGEVR WRPLVEKNPQ SILKPHAPTY NDEGL
