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CATH domain | Related DB codes (homologues) |
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2.10.25.10 : Laminin | M00133,M00212,M00152,M00155,M00315,M00316 | 2.10.70.10 : Complement Module; domain 1 | M00155,M00315,M00316 | 2.40.10.10 : Thrombin, subunit H | D00214,M00167,D00426,M00133,D00428,D00429,D00430,D00431,D00432,D00433,D00434,D00435,M00227,M00209,D00194,D00197,D00211,D00212,D00216,M00212,D00224,D00497,M00217,M00216,D00528,D00848,D00850,D00851,D00852,D00855,M00152,M00155,M00157,M00181,M00315,M00316,M00317,M00348,M00349,T00074,T00410,T00411 | 2.60.120.290 : Jelly Rolls | M00227,M00315,M00316,M00317,M00348 |
Enzyme Name | UniProtKB | KEGG |
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| P00736 |
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Protein name | Complement C1r subcomponent | complement subcomponent C_overbar_1r_activated complement C1rC_overbar_1r_ esteraseactivated complement C1r |
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Synonyms | EC 3.4.21.41Complement component 1, r subcomponent |
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Contains | Complement C1r subcomponent heavy chainComplement C1r subcomponent light chain |
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RefSeq | NP_001724.3 (Protein) NM_001733.4 (DNA/RNA sequence)
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MEROPS | S01.192 (Serine)
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Pfam | PF00431 (CUB) PF07645 (EGF_CA) PF00084 (Sushi) PF00089 (Trypsin) [Graphical view]
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UniProtKB:Accession Number | P00736 |
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Entry name | C1R_HUMAN |
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Activity | Selective cleavage of Lys(or Arg)-|-Ile bond in complement subcomponent C1s to form the active form of C1s (EC 3.4.21.42). |
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Subunit | C1 is a calcium-dependent trimolecular complex of C1q, C1r and C1s in the molar ration of 1:2:2. C1r is a dimer of identical chains, each of which is activated by cleavage into two chains, A and B, connected by disulfide bonds. |
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Subcellular location |
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Cofactor |
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References for Catalytic Mechanism | References | Sections | No. of steps in catalysis |
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[4] | p.111 |
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references | [1] |
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PubMed ID | 6281332 |
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Journal | J Immunol |
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Year | 1982 |
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Volume | 128 |
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Pages | 2500-4 |
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Authors | Ziccardi RJ |
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Title | Spontaneous activation of the first component of human complement (C1) by an intramolecular autocatalytic mechanism. |
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[2] |
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PubMed ID | 2842400 |
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Journal | J Immunol |
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Year | 1988 |
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Volume | 141 |
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Pages | 1610-4 |
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Authors | Hosoi S, Borsos T |
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Title | Activation of human C1r: Western blot analysis reveals slow and dose-dependent activation. |
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[3] |
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PubMed ID | 2539098 |
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Journal | Biochem J |
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Year | 1989 |
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Volume | 257 |
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Pages | 885-91 |
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Authors | Lacroix MB, Aude CA, Arlaud GJ, Colomb MG |
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Title | Isolation and functional characterization of the proenzyme form of the catalytic domains of human C1r. |
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[4] |
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PubMed ID | 8216203 |
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Journal | Biochem J |
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Year | 1993 |
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Volume | 295 |
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Pages | 109-14 |
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Authors | Perkins SJ, Smith KF |
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Title | Identity of the putative serine-proteinase fold in proteins of the complement system with nine relevant crystal structures. |
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[5] |
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PubMed ID | 9174342 |
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Journal | Biochemistry |
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Year | 1997 |
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Volume | 36 |
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Pages | 6270-82 |
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Authors | Lacroix M, Rossi V, Gaboriaud C, Chevallier S, Jaquinod M, Thielens NM, Gagnon J, Arlaud GJ |
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Title | Structure and assembly of the catalytic region of human complement protease C1r: a three-dimensional model based on chemical cross-linking and homology modeling. |
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[6] |
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PubMed ID | 9151255 |
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Journal | J Pept Res |
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Year | 1997 |
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Volume | 49 |
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Pages | 221-31 |
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Authors | Hernandez JF, Bersch B, Petillot Y, Gagnon J, Arlaud GJ |
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Title | Chemical synthesis and characterization of the epidermal growth factor-like module of human complement protease C1r. |
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[7] |
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Comments | STRUCTURE BY NMR OF 140-192. |
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Medline ID | 98138432 |
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PubMed ID | 9477945 |
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Journal | Biochemistry |
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Year | 1998 |
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Volume | 37 |
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Pages | 1204-14 |
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Authors | Bersch B, Hernandez JF, Marion D, Arlaud GJ |
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Title | Solution structure of the epidermal growth factor (EGF)-like module of human complement protease C1r, an atypical member of the EGF family. |
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Related PDB | 1apq |
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Related UniProtKB | P00736 |
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[8] |
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PubMed ID | 9777414 |
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Journal | Immunobiology |
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Year | 1998 |
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Volume | 199 |
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Pages | 303-16 |
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Authors | Arlaud GJ, Rossi V, Thielens NM, Gaboriaud C, Bersch B, Hernandez JF |
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Title | Structural and functional studies on C1r and C1s: new insights into the mechanisms involved in C1 activity and assembly. |
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[9] |
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PubMed ID | 9777415 |
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Journal | Immunobiology |
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Year | 1998 |
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Volume | 199 |
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Pages | 317-26 |
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Authors | Gal P, Zavodszky P |
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Title | Structure and function of the serine-protease subcomponents of C1: protein engineering studies. |
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[10] |
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PubMed ID | 11414355 |
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Journal | Immunol Rev |
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Year | 2001 |
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Volume | 180 |
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Pages | 136-45 |
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Authors | Arlaud GJ, Gaboriaud C, Thielens NM, Rossi V, Bersch B, Hernandez JF, Fontecilla-Camps JC |
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Title | Structural biology of C1: dissection of a complex molecular machinery. |
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[11] |
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PubMed ID | 11445589 |
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Journal | J Biol Chem |
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Year | 2001 |
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Volume | 276 |
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Pages | 36233-40 |
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Authors | Lacroix M, Ebel C, Kardos J, Dobo J, Gal P, Zavodszky P, Arlaud GJ, Thielens NM |
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Title | Assembly and enzymatic properties of the catalytic domain of human complement protease C1r. |
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[12] |
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PubMed ID | 11823416 |
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Journal | EMBO J |
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Year | 2002 |
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Volume | 21 |
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Pages | 231-9 |
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Authors | Budayova-Spano M, Lacroix M, Thielens NM, Arlaud GJ, Fontecilla-Camps JC, Gaboriaud C |
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Title | The crystal structure of the zymogen catalytic domain of complement protease C1r reveals that a disruptive mechanical stress is required to trigger activation of the C1 complex. |
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Related PDB | 1gpz |
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[13] |
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PubMed ID | 12429092 |
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Journal | Structure (Camb) |
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Year | 2002 |
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Volume | 10 |
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Pages | 1509-19 |
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Authors | Budayova-Spano M, Grabarse W, Thielens NM, Hillen H, Lacroix M, Schmidt M, Fontecilla-Camps JC, Arlaud GJ, Gaboriaud C |
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Title | Monomeric structures of the zymogen and active catalytic domain of complement protease c1r: further insights into the c1 activation mechanism. |
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Related PDB | 1md7,1md8 |
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comments | This enzyme belongs to the peptidase family-S1. According to the literature [4], this enzyme has got a similar catalytic triad (Ser/His/Asp) to that of trypsin, suggesting a similar mechanism.
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created | updated |
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2004-03-19 | 2012-08-08 |
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