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Q9NZP8

- C1RL_HUMAN

UniProt

Q9NZP8 - C1RL_HUMAN

Protein

Complement C1r subcomponent-like protein

Gene

C1RL

Organism
Homo sapiens (Human)
Status
Reviewed - Annotation score: 5 out of 5- Experimental evidence at protein leveli
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    • History
      Entry version 111 (01 Oct 2014)
      Sequence version 2 (26 Feb 2008)
      Previous versions | rss
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    Functioni

    Mediates the proteolytic cleavage of HP/haptoglobin in the endoplasmic reticulum.3 Publications

    Sites

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Active sitei283 – 2831Charge relay systemBy similarity
    Active sitei339 – 3391Charge relay systemBy similarity
    Active sitei436 – 4361Charge relay systemBy similarity

    GO - Molecular functioni

    1. serine-type endopeptidase activity Source: InterPro

    GO - Biological processi

    1. complement activation, classical pathway Source: UniProtKB-KW
    2. innate immune response Source: UniProtKB-KW

    Keywords - Molecular functioni

    Hydrolase, Protease, Serine protease

    Keywords - Biological processi

    Complement pathway, Immunity, Innate immunity

    Protein family/group databases

    MEROPSiS01.189.

    Names & Taxonomyi

    Protein namesi
    Recommended name:
    Complement C1r subcomponent-like protein (EC:3.4.21.-)
    Short name:
    C1r-LP
    Short name:
    C1r-like protein
    Alternative name(s):
    C1r-like serine protease analog protein
    Short name:
    CLSPa
    Gene namesi
    Name:C1RL
    Synonyms:C1RL1, C1RLP, CLSPA
    OrganismiHomo sapiens (Human)
    Taxonomic identifieri9606 [NCBI]
    Taxonomic lineageiEukaryotaMetazoaChordataCraniataVertebrataEuteleostomiMammaliaEutheriaEuarchontogliresPrimatesHaplorrhiniCatarrhiniHominidaeHomo
    ProteomesiUP000005640: Chromosome 12

    Organism-specific databases

    HGNCiHGNC:21265. C1RL.

    Subcellular locationi

    Secreted 1 Publication

    GO - Cellular componenti

    1. extracellular space Source: UniProt
    2. extracellular vesicular exosome Source: UniProt

    Keywords - Cellular componenti

    Secreted

    Pathology & Biotechi

    Mutagenesis

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Mutagenesisi436 – 4361S → A: Unable to cleave HP. 1 Publication

    Organism-specific databases

    PharmGKBiPA134957759.

    PTM / Processingi

    Molecule processing

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Signal peptidei1 – 3535Sequence AnalysisAdd
    BLAST
    Chaini36 – 487452Complement C1r subcomponent-like proteinPRO_0000318678Add
    BLAST

    Amino acid modifications

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Disulfide bondi94 ↔ 112By similarity
    Glycosylationi147 – 1471N-linked (GlcNAc...)1 Publication
    Glycosylationi166 – 1661N-linked (GlcNAc...)1 Publication
    Glycosylationi242 – 2421N-linked (GlcNAc...) (complex)3 Publications
    Glycosylationi296 – 2961N-linked (GlcNAc...)2 Publications
    Glycosylationi363 – 3631N-linked (GlcNAc...)Sequence Analysis
    Disulfide bondi402 ↔ 421By similarity
    Disulfide bondi432 ↔ 462By similarity

    Keywords - PTMi

    Disulfide bond, Glycoprotein

    Proteomic databases

    PaxDbiQ9NZP8.
    PRIDEiQ9NZP8.

    PTM databases

    PhosphoSiteiQ9NZP8.

    Expressioni

    Tissue specificityi

    Highly expressed in placenta, liver, kidney, pancreas, moderately in lung, spleen, prostate, ovary, colon, and PBL, and weakly in heart, skeletal muscle, thymus, testis, and small intestine. Expressed in PC-3 (prostate adenocarcinoma) and SK-OV-3 (ovary adenocarcinoma) cells, but not in LoVo and HT-29 (colon adenocarcinoma), SMMC7721 (hepatocellular carcinoma), CaoV-3 (ovary adenocarcinoma), HeLa (cervix epithelioid carcinoma), MCF-7 (breast adenocarcinoma), U-251MG (glioma) or A-549 (lung carcinoma) cells. Widely expressed in myeloid leukemia cell lines, including K-562 (chronic myelogenous leukemia), THP-1 (myelomonocytic leukemia), HL-60 and NB4 (promyelocytic leukemia), and KG-1 (acute myelogenous leukemia) cells. Expressed mainly in the liver and in serum (at protein level).2 Publications

    Inductioni

    Up-regulated in monocytes and dendritic cells (DC) undergoing maturation or activation.1 Publication

    Gene expression databases

    ArrayExpressiQ9NZP8.
    BgeeiQ9NZP8.
    CleanExiHS_C1RL.
    GenevestigatoriQ9NZP8.

    Organism-specific databases

    HPAiHPA011338.

    Interactioni

    Protein-protein interaction databases

    BioGridi119431. 2 interactions.
    STRINGi9606.ENSP00000266542.

    Structurei

    3D structure databases

    ProteinModelPortaliQ9NZP8.
    SMRiQ9NZP8. Positions 51-483.
    ModBaseiSearch...
    MobiDBiSearch...

    Family & Domainsi

    Domains and Repeats

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Domaini39 – 163125CUBPROSITE-ProRule annotationAdd
    BLAST
    Domaini245 – 484240Peptidase S1PROSITE-ProRule annotationAdd
    BLAST

    Sequence similaritiesi

    Belongs to the peptidase S1 family.PROSITE-ProRule annotation
    Contains 1 CUB domain.PROSITE-ProRule annotation
    Contains 1 peptidase S1 domain.PROSITE-ProRule annotation

    Keywords - Domaini

    Signal

    Phylogenomic databases

    eggNOGiCOG5640.
    HOGENOMiHOG000237311.
    HOVERGENiHBG000559.
    InParanoidiQ9NZP8.
    OMAiWQAFTSI.
    PhylomeDBiQ9NZP8.
    TreeFamiTF330373.

    Family and domain databases

    Gene3Di2.60.120.290. 1 hit.
    InterProiIPR000859. CUB_dom.
    IPR001254. Peptidase_S1.
    IPR018114. Peptidase_S1_AS.
    IPR001314. Peptidase_S1A.
    IPR000436. Sushi_SCR_CCP.
    IPR009003. Trypsin-like_Pept_dom.
    [Graphical view]
    PfamiPF00431. CUB. 1 hit.
    PF00089. Trypsin. 1 hit.
    [Graphical view]
    PRINTSiPR00722. CHYMOTRYPSIN.
    SMARTiSM00042. CUB. 1 hit.
    SM00020. Tryp_SPc. 1 hit.
    [Graphical view]
    SUPFAMiSSF49854. SSF49854. 1 hit.
    SSF50494. SSF50494. 1 hit.
    SSF57535. SSF57535. 1 hit.
    PROSITEiPS01180. CUB. 1 hit.
    PS50240. TRYPSIN_DOM. 1 hit.
    PS00135. TRYPSIN_SER. 1 hit.
    [Graphical view]

    Sequencei

    Sequence statusi: Complete.

    Sequence processingi: The displayed sequence is further processed into a mature form.

    Q9NZP8-1 [UniParc]FASTAAdd to Basket

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    MPGPRVWGKY LWRSPHSKGC PGAMWWLLLW GVLQACPTRG SVLLAQELPQ    50
    QLTSPGYPEP YGKGQESSTD IKAPEGFAVR LVFQDFDLEP SQDCAGDSVT 100
    ISFVGSDPSQ FCGQQGSPLG RPPGQREFVS SGRSLRLTFR TQPSSENKTA 150
    HLHKGFLALY QTVAVNYSQP ISEASRGSEA INAPGDNPAK VQNHCQEPYY 200
    QAAAAGALTC ATPGTWKDRQ DGEEVLQCMP VCGRPVTPIA QNQTTLGSSR 250
    AKLGNFPWQA FTSIHGRGGG ALLGDRWILT AAHTIYPKDS VSLRKNQSVN 300
    VFLGHTAIDE MLKLGNHPVH RVVVHPDYRQ NESHNFSGDI ALLELQHSIP 350
    LGPNVLPVCL PDNETLYRSG LLGYVSGFGM EMGWLTTELK YSRLPVAPRE 400
    ACNAWLQKRQ RPEVFSDNMF CVGDETQRHS VCQGDSGSVY VVWDNHAHHW 450
    VATGIVSWGI GCGEGYDFYT KVLSYVDWIK GVMNGKN 487
    Length:487
    Mass (Da):53,498
    Last modified:February 26, 2008 - v2
    Checksum:i6DE7CE9EA08C7990
    GO

    Experimental Info

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Sequence conflicti94 – 941C → R in BAD96522. 1 PublicationCurated
    Sequence conflicti99 – 991V → A in BAD96522. 1 PublicationCurated
    Sequence conflicti349 – 3491I → M in BAD96522. 1 PublicationCurated

    Natural variant

    Feature keyPosition(s)LengthDescriptionGraphical viewFeature identifierActions
    Natural varianti285 – 2851I → V.1 Publication
    Corresponds to variant rs3742089 [ dbSNP | Ensembl ].
    VAR_038852

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AF178985 mRNA. Translation: AAF44349.1.
    AK222802 mRNA. Translation: BAD96522.1.
    AC018653 Genomic DNA. No translation available.
    AC094008 Genomic DNA. No translation available.
    AC233309 Genomic DNA. No translation available.
    CCDSiCCDS8573.1.
    RefSeqiNP_057630.2. NM_016546.2.
    UniGeneiHs.631730.

    Genome annotation databases

    EnsembliENST00000266542; ENSP00000266542; ENSG00000139178.
    GeneIDi51279.
    KEGGihsa:51279.
    UCSCiuc001qsn.3. human.

    Polymorphism databases

    DMDMi182705204.

    Keywords - Coding sequence diversityi

    Polymorphism

    Cross-referencesi

    Sequence databases

    Select the link destinations:
    EMBL
    GenBank
    DDBJ
    Links Updated
    AF178985 mRNA. Translation: AAF44349.1 .
    AK222802 mRNA. Translation: BAD96522.1 .
    AC018653 Genomic DNA. No translation available.
    AC094008 Genomic DNA. No translation available.
    AC233309 Genomic DNA. No translation available.
    CCDSi CCDS8573.1.
    RefSeqi NP_057630.2. NM_016546.2.
    UniGenei Hs.631730.

    3D structure databases

    ProteinModelPortali Q9NZP8.
    SMRi Q9NZP8. Positions 51-483.
    ModBasei Search...
    MobiDBi Search...

    Protein-protein interaction databases

    BioGridi 119431. 2 interactions.
    STRINGi 9606.ENSP00000266542.

    Protein family/group databases

    MEROPSi S01.189.

    PTM databases

    PhosphoSitei Q9NZP8.

    Polymorphism databases

    DMDMi 182705204.

    Proteomic databases

    PaxDbi Q9NZP8.
    PRIDEi Q9NZP8.

    Protocols and materials databases

    DNASUi 51279.
    Structural Biology Knowledgebase Search...

    Genome annotation databases

    Ensembli ENST00000266542 ; ENSP00000266542 ; ENSG00000139178 .
    GeneIDi 51279.
    KEGGi hsa:51279.
    UCSCi uc001qsn.3. human.

    Organism-specific databases

    CTDi 51279.
    GeneCardsi GC12M007247.
    H-InvDB HIX0010393.
    HGNCi HGNC:21265. C1RL.
    HPAi HPA011338.
    MIMi 608974. gene.
    neXtProti NX_Q9NZP8.
    PharmGKBi PA134957759.
    GenAtlasi Search...

    Phylogenomic databases

    eggNOGi COG5640.
    HOGENOMi HOG000237311.
    HOVERGENi HBG000559.
    InParanoidi Q9NZP8.
    OMAi WQAFTSI.
    PhylomeDBi Q9NZP8.
    TreeFami TF330373.

    Miscellaneous databases

    ChiTaRSi C1RL. human.
    GenomeRNAii 51279.
    NextBioi 54501.
    PROi Q9NZP8.
    SOURCEi Search...

    Gene expression databases

    ArrayExpressi Q9NZP8.
    Bgeei Q9NZP8.
    CleanExi HS_C1RL.
    Genevestigatori Q9NZP8.

    Family and domain databases

    Gene3Di 2.60.120.290. 1 hit.
    InterProi IPR000859. CUB_dom.
    IPR001254. Peptidase_S1.
    IPR018114. Peptidase_S1_AS.
    IPR001314. Peptidase_S1A.
    IPR000436. Sushi_SCR_CCP.
    IPR009003. Trypsin-like_Pept_dom.
    [Graphical view ]
    Pfami PF00431. CUB. 1 hit.
    PF00089. Trypsin. 1 hit.
    [Graphical view ]
    PRINTSi PR00722. CHYMOTRYPSIN.
    SMARTi SM00042. CUB. 1 hit.
    SM00020. Tryp_SPc. 1 hit.
    [Graphical view ]
    SUPFAMi SSF49854. SSF49854. 1 hit.
    SSF50494. SSF50494. 1 hit.
    SSF57535. SSF57535. 1 hit.
    PROSITEi PS01180. CUB. 1 hit.
    PS50240. TRYPSIN_DOM. 1 hit.
    PS00135. TRYPSIN_SER. 1 hit.
    [Graphical view ]
    ProtoNeti Search...

    Publicationsi

    1. "A novel human dendritic cell-derived C1r-like serine protease analog inhibits complement-mediated cytotoxicity."
      Lin N., Liu S., Li N., Wu P., An H., Yu Y., Wan T., Cao X.
      Biochem. Biophys. Res. Commun. 321:329-336(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA], FUNCTION, SUBCELLULAR LOCATION, TISSUE SPECIFICITY, INDUCTION, VARIANT VAL-285.
      Tissue: Dendritic cell.
    2. "A novel human complement-related protein, C1r-like protease (C1r-LP), specifically cleaves pro-C1s."
      Ligoudistianou C., Xu Y., Garnier G., Circolo A., Volanakis J.E.
      Biochem. J. 387:165-173(2005) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [MRNA], FUNCTION, TISSUE SPECIFICITY.
    3. Suzuki Y., Sugano S., Totoki Y., Toyoda A., Takeda T., Sakaki Y., Tanaka A., Yokoyama S.
      Submitted (APR-2005) to the EMBL/GenBank/DDBJ databases
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE MRNA].
      Tissue: Liver.
    4. "The finished DNA sequence of human chromosome 12."
      Scherer S.E., Muzny D.M., Buhay C.J., Chen R., Cree A., Ding Y., Dugan-Rocha S., Gill R., Gunaratne P., Harris R.A., Hawes A.C., Hernandez J., Hodgson A.V., Hume J., Jackson A., Khan Z.M., Kovar-Smith C., Lewis L.R.
      , Lozado R.J., Metzker M.L., Milosavljevic A., Miner G.R., Montgomery K.T., Morgan M.B., Nazareth L.V., Scott G., Sodergren E., Song X.-Z., Steffen D., Lovering R.C., Wheeler D.A., Worley K.C., Yuan Y., Zhang Z., Adams C.Q., Ansari-Lari M.A., Ayele M., Brown M.J., Chen G., Chen Z., Clerc-Blankenburg K.P., Davis C., Delgado O., Dinh H.H., Draper H., Gonzalez-Garay M.L., Havlak P., Jackson L.R., Jacob L.S., Kelly S.H., Li L., Li Z., Liu J., Liu W., Lu J., Maheshwari M., Nguyen B.-V., Okwuonu G.O., Pasternak S., Perez L.M., Plopper F.J.H., Santibanez J., Shen H., Tabor P.E., Verduzco D., Waldron L., Wang Q., Williams G.A., Zhang J., Zhou J., Allen C.C., Amin A.G., Anyalebechi V., Bailey M., Barbaria J.A., Bimage K.E., Bryant N.P., Burch P.E., Burkett C.E., Burrell K.L., Calderon E., Cardenas V., Carter K., Casias K., Cavazos I., Cavazos S.R., Ceasar H., Chacko J., Chan S.N., Chavez D., Christopoulos C., Chu J., Cockrell R., Cox C.D., Dang M., Dathorne S.R., David R., Davis C.M., Davy-Carroll L., Deshazo D.R., Donlin J.E., D'Souza L., Eaves K.A., Egan A., Emery-Cohen A.J., Escotto M., Flagg N., Forbes L.D., Gabisi A.M., Garza M., Hamilton C., Henderson N., Hernandez O., Hines S., Hogues M.E., Huang M., Idlebird D.G., Johnson R., Jolivet A., Jones S., Kagan R., King L.M., Leal B., Lebow H., Lee S., LeVan J.M., Lewis L.C., London P., Lorensuhewa L.M., Loulseged H., Lovett D.A., Lucier A., Lucier R.L., Ma J., Madu R.C., Mapua P., Martindale A.D., Martinez E., Massey E., Mawhiney S., Meador M.G., Mendez S., Mercado C., Mercado I.C., Merritt C.E., Miner Z.L., Minja E., Mitchell T., Mohabbat F., Mohabbat K., Montgomery B., Moore N., Morris S., Munidasa M., Ngo R.N., Nguyen N.B., Nickerson E., Nwaokelemeh O.O., Nwokenkwo S., Obregon M., Oguh M., Oragunye N., Oviedo R.J., Parish B.J., Parker D.N., Parrish J., Parks K.L., Paul H.A., Payton B.A., Perez A., Perrin W., Pickens A., Primus E.L., Pu L.-L., Puazo M., Quiles M.M., Quiroz J.B., Rabata D., Reeves K., Ruiz S.J., Shao H., Sisson I., Sonaike T., Sorelle R.P., Sutton A.E., Svatek A.F., Svetz L.A., Tamerisa K.S., Taylor T.R., Teague B., Thomas N., Thorn R.D., Trejos Z.Y., Trevino B.K., Ukegbu O.N., Urban J.B., Vasquez L.I., Vera V.A., Villasana D.M., Wang L., Ward-Moore S., Warren J.T., Wei X., White F., Williamson A.L., Wleczyk R., Wooden H.S., Wooden S.H., Yen J., Yoon L., Yoon V., Zorrilla S.E., Nelson D., Kucherlapati R., Weinstock G., Gibbs R.A.
      Nature 440:346-351(2006) [PubMed] [Europe PMC] [Abstract]
      Cited for: NUCLEOTIDE SEQUENCE [LARGE SCALE GENOMIC DNA].
    5. "Prohaptoglobin is proteolytically cleaved in the endoplasmic reticulum by the complement C1r-like protein."
      Wicher K.B., Fries E.
      Proc. Natl. Acad. Sci. U.S.A. 101:14390-14395(2004) [PubMed] [Europe PMC] [Abstract]
      Cited for: FUNCTION, MUTAGENESIS OF SER-436.
    6. "Human plasma N-glycoproteome analysis by immunoaffinity subtraction, hydrazide chemistry, and mass spectrometry."
      Liu T., Qian W.-J., Gritsenko M.A., Camp D.G. II, Monroe M.E., Moore R.J., Smith R.D.
      J. Proteome Res. 4:2070-2080(2005) [PubMed] [Europe PMC] [Abstract]
      Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-166; ASN-242 AND ASN-296.
      Tissue: Plasma.
    7. "Glycoproteomics analysis of human liver tissue by combination of multiple enzyme digestion and hydrazide chemistry."
      Chen R., Jiang X., Sun D., Han G., Wang F., Ye M., Wang L., Zou H.
      J. Proteome Res. 8:651-661(2009) [PubMed] [Europe PMC] [Abstract]
      Cited for: GLYCOSYLATION [LARGE SCALE ANALYSIS] AT ASN-147; ASN-242 AND ASN-296.
      Tissue: Liver.
    8. Cited for: GLYCOSYLATION AT ASN-242.

    Entry informationi

    Entry nameiC1RL_HUMAN
    AccessioniPrimary (citable) accession number: Q9NZP8
    Secondary accession number(s): Q53GX9
    Entry historyi
    Integrated into UniProtKB/Swiss-Prot: February 26, 2008
    Last sequence update: February 26, 2008
    Last modified: October 1, 2014
    This is version 111 of the entry and version 2 of the sequence. [Complete history]
    Entry statusiReviewed (UniProtKB/Swiss-Prot)
    Annotation programChordata Protein Annotation Program
    DisclaimerAny medical or genetic information present in this entry is provided for research, educational and informational purposes only. It is not in any way intended to be used as a substitute for professional medical advice, diagnosis, treatment or care.

    Miscellaneousi

    Caution

    Does not associate with the C1 complex. According to PubMed:15385675, doesn't cleave the proform of complement C1s.Curated

    Keywords - Technical termi

    Complete proteome, Reference proteome

    Documents

    1. Human chromosome 12
      Human chromosome 12: entries, gene names and cross-references to MIM
    2. Human entries with polymorphisms or disease mutations
      List of human entries with polymorphisms or disease mutations
    3. Human polymorphisms and disease mutations
      Index of human polymorphisms and disease mutations
    4. MIM cross-references
      Online Mendelian Inheritance in Man (MIM) cross-references in UniProtKB/Swiss-Prot
    5. Peptidase families
      Classification of peptidase families and list of entries
    6. SIMILARITY comments
      Index of protein domains and families

    External Data

    Dasty 3