UBP53_MOUSE
ID UBP53_MOUSE Reviewed; 1069 AA.
AC P15975; A2RT25; Q8BR11; Q8CB37; Q8R251;
DT 01-APR-1990, integrated into UniProtKB/Swiss-Prot.
DT 07-FEB-2006, sequence version 2.
DT 03-AUG-2022, entry version 133.
DE RecName: Full=Inactive ubiquitin carboxyl-terminal hydrolase 53;
DE AltName: Full=Inactive ubiquitin-specific peptidase 53;
DE AltName: Full=Per-hexamer repeat protein 3;
DE AltName: Full=Protein mambo {ECO:0000303|PubMed:26609154};
GN Name=Usp53 {ECO:0000312|MGI:MGI:2139607}; Synonyms=Phxr3;
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=Vagina;
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=FVB/N; TISSUE=Brain, and Kidney;
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 PARTIAL NUCLEOTIDE SEQUENCE [MRNA].
RC TISSUE=Spleen;
RX PubMed=3267239; DOI=10.1093/nar/16.24.11831;
RA Nishimatsu S., Murakami K., Mitsui Y., Ishida N.;
RT "Mouse spleen derived cDNA clones containing per repeat sequence.";
RL Nucleic Acids Res. 16:11831-11832(1988).
RN [4]
RP FUNCTION, MUTAGENESIS OF CYS-228, ALTERNATIVE SPLICING, INTERACTION WITH
RP TJP1 AND TJP2, SUBCELLULAR LOCATION, AND TISSUE SPECIFICITY.
RX PubMed=26609154; DOI=10.1523/jneurosci.1965-15.2015;
RA Kazmierczak M., Harris S.L., Kazmierczak P., Shah P., Starovoytov V.,
RA Ohlemiller K.K., Schwander M.;
RT "Progressive hearing loss in mice carrying a mutation in usp53.";
RL J. Neurosci. 35:15582-15598(2015).
CC -!- FUNCTION: Tight junction-associated protein that is involved in the
CC survival of auditory hair cells and hearing. Maybe by modulating the
CC barrier properties and mechanical stability of tight junctions
CC (PubMed:26609154). Has no peptidase activity (PubMed:26609154).
CC {ECO:0000269|PubMed:26609154}.
CC -!- SUBUNIT: Interacts (via the C-terminal region) with the heterodimer
CC TJP1:TJP2. {ECO:0000269|PubMed:26609154}.
CC -!- SUBCELLULAR LOCATION: Cell junction, tight junction
CC {ECO:0000269|PubMed:26609154}.
CC -!- ALTERNATIVE PRODUCTS:
CC Event=Alternative splicing; Named isoforms=2;
CC Name=1;
CC IsoId=P15975-1; Sequence=Displayed;
CC Name=2;
CC IsoId=P15975-2; Sequence=VSP_058590, VSP_058591;
CC -!- TISSUE SPECIFICITY: Expressed in the cochlea. Isoform 1 expression
CC levels are 10-fold higher than isoform 2 expression levels.
CC {ECO:0000269|PubMed:26609154}.
CC -!- SIMILARITY: Belongs to the peptidase C19 family. {ECO:0000305}.
CC -!- CAUTION: Although the active site residues are conserved, lacks the
CC conserved His residue which is normally found 9 residues before the
CC catalytic His. {ECO:0000305}.
CC -!- SEQUENCE CAUTION:
CC Sequence=CAA31296.1; Type=Miscellaneous discrepancy; Note=Chimeric cDNA.; Evidence={ECO:0000305};
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DR EMBL; AK036876; BAC29614.1; -; mRNA.
DR EMBL; AK045953; BAC32545.1; -; mRNA.
DR EMBL; BC022221; AAH22221.1; -; mRNA.
DR EMBL; BC132339; AAI32340.1; -; mRNA.
DR EMBL; BC145707; AAI45708.1; -; mRNA.
DR EMBL; X12807; CAA31296.1; ALT_SEQ; mRNA.
DR CCDS; CCDS17815.1; -. [P15975-1]
DR PIR; S02187; S02187.
DR RefSeq; NP_598618.1; NM_133857.3. [P15975-1]
DR RefSeq; XP_011238619.1; XM_011240317.1. [P15975-1]
DR RefSeq; XP_017175309.1; XM_017319820.1. [P15975-1]
DR AlphaFoldDB; P15975; -.
DR SMR; P15975; -.
DR STRING; 10090.ENSMUSP00000087857; -.
DR MEROPS; C19.081; -.
DR iPTMnet; P15975; -.
DR PhosphoSitePlus; P15975; -.
DR MaxQB; P15975; -.
DR PaxDb; P15975; -.
DR PRIDE; P15975; -.
DR ProteomicsDB; 298421; -. [P15975-1]
DR ProteomicsDB; 298422; -. [P15975-2]
DR Antibodypedia; 26664; 113 antibodies from 23 providers.
DR DNASU; 99526; -.
DR Ensembl; ENSMUST00000090379; ENSMUSP00000087857; ENSMUSG00000039701. [P15975-1]
DR Ensembl; ENSMUST00000197314; ENSMUSP00000142600; ENSMUSG00000039701. [P15975-2]
DR GeneID; 99526; -.
DR KEGG; mmu:99526; -.
DR UCSC; uc008rez.1; mouse. [P15975-1]
DR CTD; 54532; -.
DR MGI; MGI:2139607; Usp53.
DR VEuPathDB; HostDB:ENSMUSG00000039701; -.
DR eggNOG; KOG1887; Eukaryota.
DR GeneTree; ENSGT00940000156337; -.
DR HOGENOM; CLU_287278_0_0_1; -.
DR InParanoid; P15975; -.
DR OMA; MEQCYSE; -.
DR OrthoDB; 101145at2759; -.
DR PhylomeDB; P15975; -.
DR TreeFam; TF323194; -.
DR BioGRID-ORCS; 99526; 1 hit in 71 CRISPR screens.
DR ChiTaRS; Usp53; mouse.
DR PRO; PR:P15975; -.
DR Proteomes; UP000000589; Chromosome 3.
DR RNAct; P15975; protein.
DR Bgee; ENSMUSG00000039701; Expressed in gastrula and 213 other tissues.
DR ExpressionAtlas; P15975; baseline and differential.
DR Genevisible; P15975; MM.
DR GO; GO:0005923; C:bicellular tight junction; IEA:UniProtKB-SubCell.
DR GO; GO:0005911; C:cell-cell junction; IDA:MGI.
DR GO; GO:0001508; P:action potential; IMP:MGI.
DR GO; GO:1904019; P:epithelial cell apoptotic process; IMP:MGI.
DR GO; GO:0051402; P:neuron apoptotic process; IMP:MGI.
DR GO; GO:1905584; P:outer hair cell apoptotic process; IMP:MGI.
DR GO; GO:0010996; P:response to auditory stimulus; IMP:MGI.
DR GO; GO:0007605; P:sensory perception of sound; IMP:MGI.
DR InterPro; IPR038765; Papain-like_cys_pep_sf.
DR InterPro; IPR001394; Peptidase_C19_UCH.
DR InterPro; IPR040143; USP53.
DR InterPro; IPR028889; USP_dom.
DR PANTHER; PTHR22975:SF6; PTHR22975:SF6; 1.
DR Pfam; PF00443; UCH; 1.
DR SUPFAM; SSF54001; SSF54001; 1.
DR PROSITE; PS50235; USP_3; 1.
PE 1: Evidence at protein level;
KW Alternative splicing; Cell junction; Reference proteome; Tight junction.
FT CHAIN 1..1069
FT /note="Inactive ubiquitin carboxyl-terminal hydrolase 53"
FT /id="PRO_0000080682"
FT DOMAIN 30..351
FT /note="USP"
FT REGION 414..438
FT /note="Disordered"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT REGION 486..634
FT /note="Disordered"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT REGION 646..710
FT /note="Disordered"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT REGION 805..866
FT /note="Disordered"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT REGION 946..981
FT /note="Disordered"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT REGION 1046..1069
FT /note="Disordered"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 414..428
FT /note="Polar residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 492..506
FT /note="Polar residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 524..560
FT /note="Polar residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 594..616
FT /note="Basic and acidic residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 617..632
FT /note="Polar residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 670..702
FT /note="Polar residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 805..825
FT /note="Basic and acidic residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 846..864
FT /note="Polar residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 946..972
FT /note="Polar residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 1049..1069
FT /note="Polar residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT VAR_SEQ 275..283
FT /note="LFYRVTDEN -> VSQLLSFKR (in isoform 2)"
FT /id="VSP_058590"
FT VAR_SEQ 284..1069
FT /note="Missing (in isoform 2)"
FT /id="VSP_058591"
FT MUTAGEN 228
FT /note="C->S: Associated with a progressive hearing loss;
FT outer hair cells degenerate rapidly after the first
FT postnatal week."
FT /evidence="ECO:0000269|PubMed:26609154"
FT CONFLICT 192
FT /note="E -> G (in Ref. 3; BAC32545)"
FT /evidence="ECO:0000305"
SQ SEQUENCE 1069 AA; 119255 MW; 2D35E3FE657D57C8 CRC64;
MAWVKFLRKP SGNLGKAYQA GSLLSLAPTV GLLNEPGQNS CFLNSAVQVL WQLDIFRRSL
RALTGHICQG DACIFCALKT IFAQFQHSRE KALPSDNIRH ALAESFKDEQ RFQLGLMDDA
AECFENILAR IHFHLVPNRD ADMCTSKSCV THQKFAMTLY EQCVCRSCGA SSDPLPFTEL
VRYISTTALC NEVERMMERH ERVKPEMFAE LLQAANTADD YRKCPSNCGQ KIKIRRVLMN
CPEIVTIGLV WDSEHSDLTE DVVRSLATHL YLPGLFYRVT DENATDSELH LVGMICYTSR
HYCAFAFHTK SSKWVFFDDA HVKEMGTRWK DVVSKCIRCH LQPLLLFYAN PDGTAVSTED
ALRQVVHWSH YRSGEENMGC GKPIIYKPDN SKENGFGGQT KQKENHKFQT DISSLNRSQM
QTSGRRAPVK LSHDQREKIK DISRECALKA IEQKNALSSQ RKDLEKGQRK DTGRHRDLVD
EVLASFKSGS PPASDGFRQQ GNPHLYHSQG KGPCKHDRAT HESYTSGKVI SSSKSQNLLV
PGEKTTGKAK SDSGTGYETD SSQDSRDKGG SYSSKTKSRH RGWKPMRETL NVDSVFSESE
KRQHSPRRKS DIGSRPRCSK DQSFNNWPKA NPKQKGLMTI YEDEMKQEAG SRSSLETNGK
GAEKNLSAAE SRGPGTSWQM QRTESGYESS DHVSNGSASL DSPGVEGSGA VMDVGGGKAF
SEHIKMNSHN MDSMEYISHH CKDHPEGFRK ELQDLEADDK IHELHPESHV QIKSHLIKRS
QIGETNDKLF PSASPQTLAR EHVYQTNEHK VERPDRSKCS ERHNTENSEG TGLPFHVDES
SVAGKRVDSN ETVSPSSLPS SVRTAGLKPE TGPLMFWSQQ NITEQGYSDN SLSRELTLLS
ACNADSCQMP KLHCHRSPPP LPPKKYSTAS APRLERVGLS PDVGVTEAFN TNPSSLPKHS
LSPASGPSLE GSPCMTQERD KETIQVKQLA ANSYPSSCST NSFQPDQDST SVCPNETISL
TTYFSVDSCM TDTYRLKYHQ RPKLYFPESS GHHSNNSLSQ TEQVEGSIT
