ID   PCOC2_HUMAN             Reviewed;         415 AA.
AC   Q9UKZ9; B2RCH9; D3DNG4; Q9BRH3;
DT   10-MAY-2005, integrated into UniProtKB/Swiss-Prot.
DT   01-MAY-2000, sequence version 1.
DT   27-MAR-2024, entry version 163.
DE   RecName: Full=Procollagen C-endopeptidase enhancer 2;
DE   AltName: Full=Procollagen COOH-terminal proteinase enhancer 2;
DE            Short=PCPE-2;
DE            Short=Procollagen C-proteinase enhancer 2;
DE   Flags: Precursor;
GN   Name=PCOLCE2; Synonyms=PCPE2; ORFNames=UNQ250/PRO287;
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], AND TISSUE SPECIFICITY.
RX   PubMed=10873381; DOI=10.1006/geno.2000.6229;
RA   Xu H., Acott T.S., Wirtz M.K.;
RT   "Identification and expression of a novel type I procollagen C-proteinase
RT   enhancer protein gene from the glaucoma candidate region on 3q21-q24.";
RL   Genomics 66:264-273(2000).
RN   [2]
RP   NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA], AND TISSUE SPECIFICITY.
RC   TISSUE=Osteoarthritic cartilage;
RX   PubMed=11597177; DOI=10.1053/joca.2001.0421;
RA   Kumar S., Connor J.R., Dodds R.A., Halsey W., Van Horn M., Mao J.,
RA   Sathe G.M., Mui P., Agarwal P., Badger A.M., Lee J.C., Gowen M., Lark M.W.;
RT   "Identification and initial characterization of 5000 expressed sequenced
RT   tags (ESTs) each from adult human normal and osteoarthritic cartilage cDNA
RT   libraries.";
RL   Osteoarthritis Cartilage 9:641-653(2001).
RN   [3]
RP   NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RX   PubMed=12975309; DOI=10.1101/gr.1293003;
RA   Clark H.F., Gurney A.L., Abaya E., Baker K., Baldwin D.T., Brush J.,
RA   Chen J., Chow B., Chui C., Crowley C., Currell B., Deuel B., Dowd P.,
RA   Eaton D., Foster J.S., Grimaldi C., Gu Q., Hass P.E., Heldens S., Huang A.,
RA   Kim H.S., Klimowski L., Jin Y., Johnson S., Lee J., Lewis L., Liao D.,
RA   Mark M.R., Robbie E., Sanchez C., Schoenfeld J., Seshagiri S., Simmons L.,
RA   Singh J., Smith V., Stinson J., Vagts A., Vandlen R.L., Watanabe C.,
RA   Wieand D., Woods K., Xie M.-H., Yansura D.G., Yi S., Yu G., Yuan J.,
RA   Zhang M., Zhang Z., Goddard A.D., Wood W.I., Godowski P.J., Gray A.M.;
RT   "The secreted protein discovery initiative (SPDI), a large-scale effort to
RT   identify novel human secreted and transmembrane proteins: a bioinformatics
RT   assessment.";
RL   Genome Res. 13:2265-2270(2003).
RN   [4]
RP   NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RA   Ebert L., Schick M., Neubert P., Schatten R., Henze S., Korn B.;
RT   "Cloning of human full open reading frames in Gateway(TM) system entry
RT   vector (pDONR201).";
RL   Submitted (JUN-2004) to the EMBL/GenBank/DDBJ databases.
RN   [5]
RP   NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
RC   TISSUE=Synovium;
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   [6]
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   [7]
RP   PROTEIN SEQUENCE OF 24-38.
RX   PubMed=15340161; DOI=10.1110/ps.04682504;
RA   Zhang Z., Henzel W.J.;
RT   "Signal peptide prediction based on analysis of experimentally verified
RT   cleavage sites.";
RL   Protein Sci. 13:2819-2824(2004).
RN   [8]
RP   FUNCTION, INTERACTION WITH HEPARIN AND TYPE I OR II COLLAGEN, TISSUE
RP   SPECIFICITY, AND GLYCOSYLATION.
RX   PubMed=12393877; DOI=10.1074/jbc.m209891200;
RA   Steiglitz B.M., Keene D.R., Greenspan D.S.;
RT   "PCOLCE2 encodes a functional procollagen C-proteinase enhancer (PCPE2)
RT   that is a collagen-binding protein differing in distribution of expression
RT   and post-translational modification from the previously described PCPE1.";
RL   J. Biol. Chem. 277:49820-49830(2002).
CC   -!- FUNCTION: Binds to the C-terminal propeptide of types I and II
CC       procollagens and may enhance the cleavage of that propeptide by BMP1.
CC       {ECO:0000269|PubMed:12393877}.
CC   -!- SUBUNIT: Interacts with heparin with high affinity, and type I or II
CC       collagen. {ECO:0000269|PubMed:12393877}.
CC   -!- SUBCELLULAR LOCATION: Secreted {ECO:0000305}.
CC   -!- TISSUE SPECIFICITY: Highly expressed in the heart, trabecular meshwork,
CC       pituitary gland, bladder, mammary gland, trachea and placenta and
CC       weakly expressed in the brain. Expressed in cartilage.
CC       {ECO:0000269|PubMed:10873381, ECO:0000269|PubMed:11597177,
CC       ECO:0000269|PubMed:12393877}.
CC   -!- PTM: O-glycosylated; contains sialic acid.
CC       {ECO:0000269|PubMed:12393877}.
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DR   EMBL; AF098269; AAF04621.1; -; mRNA.
DR   EMBL; AY035400; AAK63128.1; -; mRNA.
DR   EMBL; AY358557; AAQ88921.1; -; mRNA.
DR   EMBL; CR536570; CAG38807.1; -; mRNA.
DR   EMBL; AK315121; BAG37576.1; -; mRNA.
DR   EMBL; CH471052; EAW78961.1; -; Genomic_DNA.
DR   EMBL; CH471052; EAW78962.1; -; Genomic_DNA.
DR   CCDS; CCDS3127.1; -.
DR   RefSeq; NP_037495.1; NM_013363.3.
DR   AlphaFoldDB; Q9UKZ9; -.
DR   SMR; Q9UKZ9; -.
DR   BioGRID; 117746; 29.
DR   IntAct; Q9UKZ9; 9.
DR   STRING; 9606.ENSP00000295992; -.
DR   GlyCosmos; Q9UKZ9; 2 sites, 2 glycans.
DR   GlyGen; Q9UKZ9; 2 sites, 2 O-linked glycans (1 site).
DR   iPTMnet; Q9UKZ9; -.
DR   PhosphoSitePlus; Q9UKZ9; -.
DR   BioMuta; PCOLCE2; -.
DR   DMDM; 67470587; -.
DR   jPOST; Q9UKZ9; -.
DR   MassIVE; Q9UKZ9; -.
DR   MaxQB; Q9UKZ9; -.
DR   PaxDb; 9606-ENSP00000295992; -.
DR   PeptideAtlas; Q9UKZ9; -.
DR   ProteomicsDB; 84920; -.
DR   Antibodypedia; 2145; 164 antibodies from 23 providers.
DR   DNASU; 26577; -.
DR   Ensembl; ENST00000295992.8; ENSP00000295992.3; ENSG00000163710.9.
DR   GeneID; 26577; -.
DR   KEGG; hsa:26577; -.
DR   MANE-Select; ENST00000295992.8; ENSP00000295992.3; NM_013363.4; NP_037495.1.
DR   UCSC; uc003evd.4; human.
DR   AGR; HGNC:8739; -.
DR   CTD; 26577; -.
DR   DisGeNET; 26577; -.
DR   GeneCards; PCOLCE2; -.
DR   HGNC; HGNC:8739; PCOLCE2.
DR   HPA; ENSG00000163710; Tissue enhanced (adipose tissue, heart muscle).
DR   MIM; 607064; gene.
DR   neXtProt; NX_Q9UKZ9; -.
DR   OpenTargets; ENSG00000163710; -.
DR   PharmGKB; PA33084; -.
DR   VEuPathDB; HostDB:ENSG00000163710; -.
DR   eggNOG; ENOG502QRMG; Eukaryota.
DR   GeneTree; ENSGT00940000157649; -.
DR   HOGENOM; CLU_034096_0_0_1; -.
DR   InParanoid; Q9UKZ9; -.
DR   OMA; TMWRTCS; -.
DR   OrthoDB; 5355450at2759; -.
DR   PhylomeDB; Q9UKZ9; -.
DR   TreeFam; TF316506; -.
DR   PathwayCommons; Q9UKZ9; -.
DR   Reactome; R-HSA-1650814; Collagen biosynthesis and modifying enzymes.
DR   SignaLink; Q9UKZ9; -.
DR   BioGRID-ORCS; 26577; 10 hits in 1145 CRISPR screens.
DR   ChiTaRS; PCOLCE2; human.
DR   GeneWiki; PCOLCE2; -.
DR   GenomeRNAi; 26577; -.
DR   Pharos; Q9UKZ9; Tbio.
DR   PRO; PR:Q9UKZ9; -.
DR   Proteomes; UP000005640; Chromosome 3.
DR   RNAct; Q9UKZ9; Protein.
DR   Bgee; ENSG00000163710; Expressed in calcaneal tendon and 173 other cell types or tissues.
DR   ExpressionAtlas; Q9UKZ9; baseline and differential.
DR   GO; GO:0005576; C:extracellular region; IEA:UniProtKB-SubCell.
DR   GO; GO:0005518; F:collagen binding; IDA:MGI.
DR   GO; GO:0008201; F:heparin binding; IDA:MGI.
DR   GO; GO:0016504; F:peptidase activator activity; IDA:MGI.
DR   GO; GO:1990830; P:cellular response to leukemia inhibitory factor; IEA:Ensembl.
DR   CDD; cd00041; CUB; 2.
DR   CDD; cd03576; NTR_PCOLCE; 1.
DR   Gene3D; 2.40.50.120; -; 1.
DR   Gene3D; 2.60.120.290; Spermadhesin, CUB domain; 2.
DR   InterPro; IPR000859; CUB_dom.
DR   InterPro; IPR001134; Netrin_domain.
DR   InterPro; IPR018933; Netrin_module_non-TIMP.
DR   InterPro; IPR035814; NTR_PCOLCE.
DR   InterPro; IPR035914; Sperma_CUB_dom_sf.
DR   InterPro; IPR008993; TIMP-like_OB-fold.
DR   PANTHER; PTHR24251; OVOCHYMASE-RELATED; 1.
DR   PANTHER; PTHR24251:SF31; PROCOLLAGEN C-ENDOPEPTIDASE ENHANCER 2; 1.
DR   Pfam; PF00431; CUB; 2.
DR   Pfam; PF01759; NTR; 1.
DR   SMART; SM00643; C345C; 1.
DR   SMART; SM00042; CUB; 2.
DR   SUPFAM; SSF49854; Spermadhesin, CUB domain; 2.
DR   SUPFAM; SSF50242; TIMP-like; 1.
DR   PROSITE; PS01180; CUB; 2.
DR   PROSITE; PS50189; NTR; 1.
DR   Genevisible; Q9UKZ9; HS.
PE   1: Evidence at protein level;
KW   Direct protein sequencing; Disulfide bond; Glycoprotein; Heparin-binding;
KW   Reference proteome; Repeat; Secreted; Signal.
FT   SIGNAL          1..23
FT                   /evidence="ECO:0000269|PubMed:15340161"
FT   CHAIN           24..415
FT                   /note="Procollagen C-endopeptidase enhancer 2"
FT                   /id="PRO_0000022020"
FT   DOMAIN          33..144
FT                   /note="CUB 1"
FT                   /evidence="ECO:0000255|PROSITE-ProRule:PRU00059"
FT   DOMAIN          154..268
FT                   /note="CUB 2"
FT                   /evidence="ECO:0000255|PROSITE-ProRule:PRU00059"
FT   DOMAIN          297..415
FT                   /note="NTR"
FT                   /evidence="ECO:0000255|PROSITE-ProRule:PRU00295"
FT   CARBOHYD        355
FT                   /note="N-linked (GlcNAc...) asparagine"
FT                   /evidence="ECO:0000255"
FT   DISULFID        33..59
FT                   /evidence="ECO:0000255|PROSITE-ProRule:PRU00059"
FT   DISULFID        86..107
FT                   /evidence="ECO:0000255|PROSITE-ProRule:PRU00059"
FT   DISULFID        154..181
FT                   /evidence="ECO:0000255|PROSITE-ProRule:PRU00059"
FT   DISULFID        208..231
FT                   /evidence="ECO:0000255|PROSITE-ProRule:PRU00059"
FT   DISULFID        297..364
FT                   /evidence="ECO:0000250|UniProtKB:Q15113"
FT   DISULFID        301..367
FT                   /evidence="ECO:0000250|UniProtKB:Q15113"
FT   DISULFID        312..415
FT                   /evidence="ECO:0000250|UniProtKB:Q15113"
FT   VARIANT         280
FT                   /note="V -> A (in dbSNP:rs35692900)"
FT                   /id="VAR_051264"
FT   VARIANT         292
FT                   /note="P -> T (in dbSNP:rs17554211)"
FT                   /id="VAR_022448"
SQ   SEQUENCE   415 AA;  45717 MW;  B9AA87FD9AAF7A5B CRC64;
     MRGANAWAPL CLLLAAATQL SRQQSPERPV FTCGGILTGE SGFIGSEGFP GVYPPNSKCT
     WKITVPEGKV VVLNFRFIDL ESDNLCRYDF VDVYNGHANG QRIGRFCGTF RPGALVSSGN
     KMMVQMISDA NTAGNGFMAM FSAAEPNERG DQYCGGLLDR PSGSFKTPNW PDRDYPAGVT
     CVWHIVAPKN QLIELKFEKF DVERDNYCRY DYVAVFNGGE VNDARRIGKY CGDSPPAPIV
     SERNELLIQF LSDLSLTADG FIGHYIFRPK KLPTTTEQPV TTTFPVTTGL KPTVALCQQK
     CRRTGTLEGN YCSSDFVLAG TVITTITRDG SLHATVSIIN IYKEGNLAIQ QAGKNMSARL
     TVVCKQCPLL RRGLNYIIMG QVGEDGRGKI MPNSFIMMFK TKNQKLLDAL KNKQC
//