ZN300_HUMAN
ID ZN300_HUMAN Reviewed; 604 AA.
AC Q96RE9; A8MY91; B3KU35; B4DU78; F5GWS1; Q06DQ3; Q17RP3; Q5H9N5;
DT 23-JAN-2002, integrated into UniProtKB/Swiss-Prot.
DT 01-DEC-2001, sequence version 1.
DT 03-AUG-2022, entry version 182.
DE RecName: Full=Zinc finger protein 300;
GN Name=ZNF300;
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 [MRNA] (ISOFORM 1), FUNCTION, SUBCELLULAR LOCATION, AND
RP TISSUE SPECIFICITY.
RX PubMed=14746915; DOI=10.1016/j.bbaexp.2003.11.011;
RA Gou D.-M., Wang J., Gao L., Sun Y., Peng X., Huang J., Li W.-X.;
RT "Identification and functional analysis of a novel human KRAB/C2H2 zinc
RT finger gene ZNF300.";
RL Biochim. Biophys. Acta 1676:203-209(2004).
RN [2]
RP NUCLEOTIDE SEQUENCE [MRNA] (ISOFORM 2), AND TISSUE SPECIFICITY.
RC TISSUE=Fetal brain;
RX PubMed=17541838; DOI=10.1080/10425170701243393;
RA Cao Y., Li J.-X., Ji C.-N., Xu X.-W., Wu M.;
RT "Molecular cloning and characterization of a novel splice variant of human
RT ZNF300 gene, which expressed highly in testis.";
RL DNA Seq. 18:312-315(2007).
RN [3]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] (ISOFORMS 1 AND 3), AND NUCLEOTIDE
RP SEQUENCE [LARGE SCALE MRNA] OF 1-38 (ISOFORM 4).
RC TISSUE=Brain, Prostate, and Testis;
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 [4]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
RX PubMed=15372022; DOI=10.1038/nature02919;
RA Schmutz J., Martin J., Terry A., Couronne O., Grimwood J., Lowry S.,
RA Gordon L.A., Scott D., Xie G., Huang W., Hellsten U., Tran-Gyamfi M.,
RA She X., Prabhakar S., Aerts A., Altherr M., Bajorek E., Black S.,
RA Branscomb E., Caoile C., Challacombe J.F., Chan Y.M., Denys M.,
RA Detter J.C., Escobar J., Flowers D., Fotopulos D., Glavina T., Gomez M.,
RA Gonzales E., Goodstein D., Grigoriev I., Groza M., Hammon N., Hawkins T.,
RA Haydu L., Israni S., Jett J., Kadner K., Kimball H., Kobayashi A.,
RA Lopez F., Lou Y., Martinez D., Medina C., Morgan J., Nandkeshwar R.,
RA Noonan J.P., Pitluck S., Pollard M., Predki P., Priest J., Ramirez L.,
RA Retterer J., Rodriguez A., Rogers S., Salamov A., Salazar A., Thayer N.,
RA Tice H., Tsai M., Ustaszewska A., Vo N., Wheeler J., Wu K., Yang J.,
RA Dickson M., Cheng J.-F., Eichler E.E., Olsen A., Pennacchio L.A.,
RA Rokhsar D.S., Richardson P., Lucas S.M., Myers R.M., Rubin E.M.;
RT "The DNA sequence and comparative analysis of human chromosome 5.";
RL Nature 431:268-274(2004).
RN [5]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
RA Mural R.J., Istrail S., Sutton G.G., Florea L., Halpern A.L., Mobarry C.M.,
RA Lippert R., Walenz B., Shatkay H., Dew I., Miller J.R., Flanigan M.J.,
RA Edwards N.J., Bolanos R., Fasulo D., Halldorsson B.V., Hannenhalli S.,
RA Turner R., Yooseph S., Lu F., Nusskern D.R., Shue B.C., Zheng X.H.,
RA Zhong F., Delcher A.L., Huson D.H., Kravitz S.A., Mouchard L., Reinert K.,
RA Remington K.A., Clark A.G., Waterman M.S., Eichler E.E., Adams M.D.,
RA Hunkapiller M.W., Myers E.W., Venter J.C.;
RL Submitted (SEP-2005) to the EMBL/GenBank/DDBJ databases.
RN [6]
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).
RN [7]
RP NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA] OF 89-604 (ISOFORMS 1/2/3/4).
RC TISSUE=Fetal brain;
RX PubMed=17974005; DOI=10.1186/1471-2164-8-399;
RA Bechtel S., Rosenfelder H., Duda A., Schmidt C.P., Ernst U.,
RA Wellenreuther R., Mehrle A., Schuster C., Bahr A., Bloecker H., Heubner D.,
RA Hoerlein A., Michel G., Wedler H., Koehrer K., Ottenwaelder B., Poustka A.,
RA Wiemann S., Schupp I.;
RT "The full-ORF clone resource of the German cDNA consortium.";
RL BMC Genomics 8:399-399(2007).
RN [8]
RP INTERACTION WITH ZNF268, AND SUBCELLULAR LOCATION.
RX PubMed=23665872; DOI=10.1007/s00018-013-1359-4;
RA Wang W., Cai J., Wu Y., Hu L., Chen Z., Hu J., Chen Z., Li W., Guo M.,
RA Huang Z.;
RT "Novel activity of KRAB domain that functions to reinforce nuclear
RT localization of KRAB-containing zinc finger proteins by interacting with
RT KAP1.";
RL Cell. Mol. Life Sci. 70:3947-3958(2013).
CC -!- FUNCTION: Has a transcriptional repressor activity.
CC {ECO:0000269|PubMed:14746915}.
CC -!- SUBUNIT: Interacts (via the KRAB domain) with TRIM28 (via the RBCC
CC domain). {ECO:0000269|PubMed:23665872}.
CC -!- INTERACTION:
CC Q96RE9-3; Q15699: ALX1; NbExp=3; IntAct=EBI-12902696, EBI-750671;
CC -!- SUBCELLULAR LOCATION: Nucleus {ECO:0000269|PubMed:14746915,
CC ECO:0000269|PubMed:23665872}.
CC -!- ALTERNATIVE PRODUCTS:
CC Event=Alternative splicing; Named isoforms=4;
CC Name=1;
CC IsoId=Q96RE9-1; Sequence=Displayed;
CC Name=2; Synonyms=Zinc finger protein 300-B, ZNF300B;
CC IsoId=Q96RE9-2; Sequence=VSP_036830;
CC Name=3;
CC IsoId=Q96RE9-3; Sequence=VSP_036831;
CC Name=4;
CC IsoId=Q96RE9-4; Sequence=VSP_047506;
CC -!- TISSUE SPECIFICITY: Expressed mostly in heart, skeletal muscle and
CC brain. Isoform 1 and isoform 2 are highly expressed in testis.
CC {ECO:0000269|PubMed:14746915, ECO:0000269|PubMed:17541838}.
CC -!- SIMILARITY: Belongs to the krueppel C2H2-type zinc-finger protein
CC family. {ECO:0000305}.
CC -!- SEQUENCE CAUTION:
CC Sequence=EAW61702.1; Type=Erroneous gene model prediction; Evidence={ECO:0000305};
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DR EMBL; AF395541; AAK83888.1; -; mRNA.
DR EMBL; DQ908918; ABI81769.1; -; mRNA.
DR EMBL; AK096465; BAG53297.1; -; mRNA.
DR EMBL; AK300528; BAG62240.1; -; mRNA.
DR EMBL; DB094933; -; NOT_ANNOTATED_CDS; mRNA.
DR EMBL; AC010441; -; NOT_ANNOTATED_CDS; Genomic_DNA.
DR EMBL; CH471062; EAW61702.1; ALT_SEQ; Genomic_DNA.
DR EMBL; BC117248; AAI17249.1; -; mRNA.
DR EMBL; CR933727; CAI46270.1; -; mRNA.
DR CCDS; CCDS4311.2; -. [Q96RE9-1]
DR CCDS; CCDS54939.1; -. [Q96RE9-4]
DR CCDS; CCDS54940.1; -. [Q96RE9-3]
DR RefSeq; NP_001166302.1; NM_001172831.1. [Q96RE9-3]
DR RefSeq; NP_001166303.1; NM_001172832.1. [Q96RE9-4]
DR RefSeq; NP_443092.1; NM_052860.2. [Q96RE9-1]
DR AlphaFoldDB; Q96RE9; -.
DR SMR; Q96RE9; -.
DR BioGRID; 124898; 4.
DR IntAct; Q96RE9; 4.
DR MINT; Q96RE9; -.
DR STRING; 9606.ENSP00000397178; -.
DR iPTMnet; Q96RE9; -.
DR PhosphoSitePlus; Q96RE9; -.
DR BioMuta; ZNF300; -.
DR DMDM; 20141015; -.
DR jPOST; Q96RE9; -.
DR MassIVE; Q96RE9; -.
DR MaxQB; Q96RE9; -.
DR PaxDb; Q96RE9; -.
DR PeptideAtlas; Q96RE9; -.
DR PRIDE; Q96RE9; -.
DR ProteomicsDB; 24200; -.
DR ProteomicsDB; 77954; -. [Q96RE9-1]
DR ProteomicsDB; 77955; -. [Q96RE9-2]
DR ProteomicsDB; 77956; -. [Q96RE9-3]
DR Antibodypedia; 28103; 121 antibodies from 18 providers.
DR DNASU; 91975; -.
DR Ensembl; ENST00000274599.10; ENSP00000274599.5; ENSG00000145908.13. [Q96RE9-1]
DR Ensembl; ENST00000418587.6; ENSP00000392593.2; ENSG00000145908.13. [Q96RE9-4]
DR Ensembl; ENST00000446148.6; ENSP00000397178.2; ENSG00000145908.13. [Q96RE9-3]
DR GeneID; 91975; -.
DR KEGG; hsa:91975; -.
DR MANE-Select; ENST00000274599.10; ENSP00000274599.5; NM_052860.4; NP_443092.1.
DR UCSC; uc021yfx.1; human. [Q96RE9-1]
DR CTD; 91975; -.
DR DisGeNET; 91975; -.
DR GeneCards; ZNF300; -.
DR HGNC; HGNC:13091; ZNF300.
DR HPA; ENSG00000145908; Low tissue specificity.
DR MIM; 612429; gene.
DR neXtProt; NX_Q96RE9; -.
DR OpenTargets; ENSG00000145908; -.
DR PharmGKB; PA37666; -.
DR VEuPathDB; HostDB:ENSG00000145908; -.
DR eggNOG; KOG1721; Eukaryota.
DR GeneTree; ENSGT00940000162678; -.
DR HOGENOM; CLU_002678_44_5_1; -.
DR InParanoid; Q96RE9; -.
DR OMA; NQCGNIF; -.
DR OrthoDB; 1318335at2759; -.
DR PhylomeDB; Q96RE9; -.
DR PathwayCommons; Q96RE9; -.
DR Reactome; R-HSA-212436; Generic Transcription Pathway.
DR SignaLink; Q96RE9; -.
DR BioGRID-ORCS; 91975; 12 hits in 1098 CRISPR screens.
DR GeneWiki; ZNF300; -.
DR GenomeRNAi; 91975; -.
DR Pharos; Q96RE9; Tbio.
DR PRO; PR:Q96RE9; -.
DR Proteomes; UP000005640; Chromosome 5.
DR RNAct; Q96RE9; protein.
DR Bgee; ENSG00000145908; Expressed in cortical plate and 121 other tissues.
DR ExpressionAtlas; Q96RE9; baseline and differential.
DR Genevisible; Q96RE9; HS.
DR GO; GO:0016604; C:nuclear body; IDA:HPA.
DR GO; GO:0005730; C:nucleolus; IDA:HPA.
DR GO; GO:0005654; C:nucleoplasm; IDA:HPA.
DR GO; GO:0005634; C:nucleus; IDA:UniProtKB.
DR GO; GO:0001228; F:DNA-binding transcription activator activity, RNA polymerase II-specific; IDA:NTNU_SB.
DR GO; GO:0000981; F:DNA-binding transcription factor activity, RNA polymerase II-specific; IBA:GO_Central.
DR GO; GO:0046872; F:metal ion binding; IEA:UniProtKB-KW.
DR GO; GO:0000978; F:RNA polymerase II cis-regulatory region sequence-specific DNA binding; IBA:GO_Central.
DR GO; GO:0043565; F:sequence-specific DNA binding; IDA:NTNU_SB.
DR GO; GO:0045944; P:positive regulation of transcription by RNA polymerase II; IDA:NTNU_SB.
DR GO; GO:0006357; P:regulation of transcription by RNA polymerase II; IBA:GO_Central.
DR CDD; cd07765; KRAB_A-box; 1.
DR InterPro; IPR001909; KRAB.
DR InterPro; IPR036051; KRAB_dom_sf.
DR InterPro; IPR036236; Znf_C2H2_sf.
DR InterPro; IPR013087; Znf_C2H2_type.
DR Pfam; PF01352; KRAB; 1.
DR Pfam; PF00096; zf-C2H2; 12.
DR SMART; SM00349; KRAB; 1.
DR SMART; SM00355; ZnF_C2H2; 12.
DR SUPFAM; SSF109640; SSF109640; 1.
DR SUPFAM; SSF57667; SSF57667; 7.
DR PROSITE; PS50805; KRAB; 1.
DR PROSITE; PS00028; ZINC_FINGER_C2H2_1; 12.
DR PROSITE; PS50157; ZINC_FINGER_C2H2_2; 12.
PE 1: Evidence at protein level;
KW Alternative splicing; DNA-binding; Metal-binding; Nucleus;
KW Reference proteome; Repeat; Repressor; Transcription;
KW Transcription regulation; Zinc; Zinc-finger.
FT CHAIN 1..604
FT /note="Zinc finger protein 300"
FT /id="PRO_0000047521"
FT DOMAIN 8..79
FT /note="KRAB"
FT /evidence="ECO:0000255|PROSITE-ProRule:PRU00119"
FT ZN_FING 269..291
FT /note="C2H2-type 1"
FT /evidence="ECO:0000255|PROSITE-ProRule:PRU00042"
FT ZN_FING 297..319
FT /note="C2H2-type 2"
FT /evidence="ECO:0000255|PROSITE-ProRule:PRU00042"
FT ZN_FING 325..347
FT /note="C2H2-type 3"
FT /evidence="ECO:0000255|PROSITE-ProRule:PRU00042"
FT ZN_FING 353..375
FT /note="C2H2-type 4"
FT /evidence="ECO:0000255|PROSITE-ProRule:PRU00042"
FT ZN_FING 381..403
FT /note="C2H2-type 5"
FT /evidence="ECO:0000255|PROSITE-ProRule:PRU00042"
FT ZN_FING 409..431
FT /note="C2H2-type 6"
FT /evidence="ECO:0000255|PROSITE-ProRule:PRU00042"
FT ZN_FING 437..459
FT /note="C2H2-type 7"
FT /evidence="ECO:0000255|PROSITE-ProRule:PRU00042"
FT ZN_FING 465..487
FT /note="C2H2-type 8"
FT /evidence="ECO:0000255|PROSITE-ProRule:PRU00042"
FT ZN_FING 493..515
FT /note="C2H2-type 9"
FT /evidence="ECO:0000255|PROSITE-ProRule:PRU00042"
FT ZN_FING 521..543
FT /note="C2H2-type 10"
FT /evidence="ECO:0000255|PROSITE-ProRule:PRU00042"
FT ZN_FING 549..571
FT /note="C2H2-type 11"
FT /evidence="ECO:0000255|PROSITE-ProRule:PRU00042"
FT ZN_FING 577..599
FT /note="C2H2-type 12"
FT /evidence="ECO:0000255|PROSITE-ProRule:PRU00042"
FT REGION 203..234
FT /note="Disordered"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT COMPBIAS 203..232
FT /note="Polar residues"
FT /evidence="ECO:0000256|SAM:MobiDB-lite"
FT VAR_SEQ 1..36
FT /note="Missing (in isoform 4)"
FT /evidence="ECO:0000303|PubMed:14702039"
FT /id="VSP_047506"
FT VAR_SEQ 1
FT /note="M -> MVPAETSSSGLLEEQK (in isoform 2)"
FT /evidence="ECO:0000303|PubMed:17541838"
FT /id="VSP_036830"
FT VAR_SEQ 1
FT /note="M -> MVPAETSSSGLLEEQKM (in isoform 3)"
FT /evidence="ECO:0000303|PubMed:14702039"
FT /id="VSP_036831"
FT VARIANT 336
FT /note="Q -> H (in dbSNP:rs1988688)"
FT /id="VAR_012355"
FT CONFLICT 253
FT /note="T -> I (in Ref. 7; CAI46270)"
FT /evidence="ECO:0000305"
FT CONFLICT 535
FT /note="H -> Q (in Ref. 7; CAI46270)"
FT /evidence="ECO:0000305"
SQ SEQUENCE 604 AA; 68743 MW; EABCF821E9FAC441 CRC64;
MMKSQGLVSF KDVAVDFTQE EWQQLDPSQR TLYRDVMLEN YSHLVSMGYP VSKPDVISKL
EQGEEPWIIK GDISNWIYPD EYQADGRQDR KSNLHNSQSC ILGTVSFHHK ILKGVTRDGS
LCSILKVCQG DGQLQRFLEN QDKLFRQVTF VNSKTVTEAS GHKYNPLGKI FQECIETDIS
IQRFHKYDAF KKNLKPNIDL PSCYKSNSRK KPDQSFGGGK SSSQSEPNSN LEKIHNGVIP
FDDNQCGNVF RNTQSLIQYQ NVETKEKSCV CVTCGKAFAK KSQLIVHQRI HTGKKPYDCG
ACGKAFSEKF HLVVHQRTHT GEKPYDCSEC GKAFSQKSSL IIHQRVHTGE KPYECSECGK
AFSQKSPLII HQRIHTGEKP YECRECGKAF SQKSQLIIHH RAHTGEKPYE CTECGKAFCE
KSHLIIHKRI HTGEKPYKCA QCEEAFSRKT ELITHQLVHT GEKPYECTEC GKTFSRKSQL
IIHQRTHTGE KPYKCSECGK AFCQKSHLIG HQRIHTGEKP YICTECGKAF SQKSHLPGHQ
RIHTGEKPYI CAECGKAFSQ KSDLVLHQRI HTGERPYQCA ICGKAFIQKS QLTVHQRIHT
VVKS
