DB code: M00139
| RLCP classification | 1.13.30000.10 : Hydrolysis | |
|---|---|---|
| CATH domain | 2.60.120.290 : Jelly Rolls | |
| 2.10.25.10 : Laminin | ||
| 2.60.120.290 : Jelly Rolls | ||
| 2.10.70.10 : Complement Module; domain 1 | ||
| 2.10.70.10 : Complement Module; domain 1 | ||
| 2.40.10.10 : Thrombin, subunit H | Catalytic domain | |
| 2.40.10.10 : Thrombin, subunit H | Catalytic domain | |
| E.C. | 3.4.21.41 | |
| CSA | ||
| M-CSA | ||
| MACiE | ||
| CATH domain | Related DB codes (homologues) |
|---|---|
| 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 |
| Uniprot Enzyme Name | UniprotKB | Protein name | Synonyms | Contains | RefSeq | MEROPS | Pfam |
|---|---|---|---|---|---|---|
| P00736 |
Complement C1r subcomponent
|
EC
3.4.21.41
Complement component 1, r subcomponent |
Complement C1r subcomponent heavy chain
Complement C1r subcomponent light chain |
NP_001724.3
(Protein)
NM_001733.4 (DNA/RNA sequence) |
S01.192
(Serine)
|
PF00431
(CUB)
PF07645 (EGF_CA) PF00084 (Sushi) PF00089 (Trypsin) [Graphical View] |
| KEGG enzyme name |
|---|
|
complement subcomponent C_overbar_1r_
activated complement C1r C_overbar_1r_ esterase activated complement C1r |
| UniprotKB: Accession Number | Entry name | Activity | Subunit | Subcellular location | Cofactor |
|---|---|---|---|---|---|
| P00736 | C1R_HUMAN | Selective cleavage of Lys(or Arg)-|-Ile bond in complement subcomponent C1s to form the active form of C1s (EC 3.4.21.42). | 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. |
| KEGG Pathways | Map code | Pathways | E.C. |
|---|
| Compound table | |||||||||||
|---|---|---|---|---|---|---|---|---|---|---|---|
| Substrates | Products | Intermediates | |||||||||
| KEGG-id | L00087 | C00001 | L00088 | I00087 | I00085 | I00086 | |||||
| E.C. | |||||||||||
| Compound | Complement subcomponent C1s | H2O | Complement subcomponent C1s- | Peptidyl-Ser-tetrahedral-intermediate (with previous peptide) | Acyl-enzyme(Peptidyl-Ser-acyl group) | Peptidyl-Ser-tetrahedral-intermediate | |||||
| Type | peptide/protein | H2O | peptide/protein | ||||||||
| ChEBI |
15377 15377 |
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| PubChem |
22247451 962 22247451 962 |
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| 1apqA |
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Unbound | Unbound | ||||
| 1gpzA04 |
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Unbound | Unbound | ||||
| 1gpzB04 |
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Unbound | Unbound | ||||
| 1gpzA03 |
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Unbound | Unbound | ||||
| 1gpzB03 |
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Unbound | Unbound | ||||
| 1md7A03 |
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Unbound | Unbound | ||||
| 1md8A03 |
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Unbound | Unbound | ||||
| 1gpzA01 |
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Unbound | Unbound | ||||
| 1gpzB01 |
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Unbound | Unbound | ||||
| 1md7A01 |
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Unbound | Unbound | ||||
| 1md8A01 |
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Unbound | Unbound | ||||
| 1gpzA02 |
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Unbound | Unbound | ||||
| 1gpzB02 |
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Unbound | Unbound | ||||
| 1md7A02 |
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Unbound | Unbound | ||||
| 1md8A02 |
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Unbound | Unbound | ||||
| Reference for Active-site residues | ||
|---|---|---|
| resource | references | E.C. |
| Swiss-prot & literature [4] | ||
| Active-site residues | ||||||||||
|---|---|---|---|---|---|---|---|---|---|---|
| PDB | Catalytic residues | Cofactor-binding residues | Modified residues | Main-chain involved in catalysis | Comment | |||||
| 1apqA |
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| 1gpzA04 |
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| 1gpzB04 |
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| 1gpzA03 |
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| 1gpzB03 |
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| 1md7A03 |
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| 1md8A03 |
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| 1gpzA01 |
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HIS 485;ASP 540 | ||||
| 1gpzB01 |
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HIS 485;ASP 540 | ||||
| 1md7A01 |
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HIS 485;ASP 540 | ||||
| 1md8A01 |
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HIS 485;ASP 540 | ||||
| 1gpzA02 |
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|
SER 637 | GLY 635;SER 637 | |||
| 1gpzB02 |
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SER 637 | GLY 635;SER 637 | |||
| 1md7A02 |
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GLY 635; | mutant S637A | |||
| 1md8A02 |
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SER 637 | GLY 635;SER 637 | |||
| References for Catalytic Mechanism | ||
|---|---|---|
| References | Sections | No. of steps in catalysis |
|
[4]
|
p.111 | |
| References | |
|---|---|
| [1] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 6281332 |
| Journal | J Immunol |
| Year | 1982 |
| Volume | 128 |
| Pages | 2500-4 |
| Authors | Ziccardi RJ |
| Title | Spontaneous activation of the first component of human complement (C1) by an intramolecular autocatalytic mechanism. |
| Related PDB | |
| Related UniProtKB | |
| [2] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 2842400 |
| Journal | J Immunol |
| Year | 1988 |
| Volume | 141 |
| Pages | 1610-4 |
| Authors | Hosoi S, Borsos T |
| Title | Activation of human C1r: Western blot analysis reveals slow and dose-dependent activation. |
| Related PDB | |
| Related UniProtKB | |
| [3] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 2539098 |
| Journal | Biochem J |
| Year | 1989 |
| Volume | 257 |
| Pages | 885-91 |
| Authors | Lacroix MB, Aude CA, Arlaud GJ, Colomb MG |
| Title | Isolation and functional characterization of the proenzyme form of the catalytic domains of human C1r. |
| Related PDB | |
| Related UniProtKB | |
| [4] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 8216203 |
| Journal | Biochem J |
| Year | 1993 |
| Volume | 295 |
| Pages | 109-14 |
| Authors | Perkins SJ, Smith KF |
| Title | Identity of the putative serine-proteinase fold in proteins of the complement system with nine relevant crystal structures. |
| Related PDB | |
| Related UniProtKB | |
| [5] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 9174342 |
| Journal | Biochemistry |
| Year | 1997 |
| Volume | 36 |
| Pages | 6270-82 |
| Authors | Lacroix M, Rossi V, Gaboriaud C, Chevallier S, Jaquinod M, Thielens NM, Gagnon J, Arlaud GJ |
| 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. |
| Related PDB | |
| Related UniProtKB | |
| [6] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 9151255 |
| Journal | J Pept Res |
| Year | 1997 |
| Volume | 49 |
| Pages | 221-31 |
| Authors | Hernandez JF, Bersch B, Petillot Y, Gagnon J, Arlaud GJ |
| Title | Chemical synthesis and characterization of the epidermal growth factor-like module of human complement protease C1r. |
| Related PDB | |
| Related UniProtKB | |
| [7] | |
| Resource | |
| Comments | STRUCTURE BY NMR OF 140-192. |
| Medline ID | 98138432 |
| PubMed ID | 9477945 |
| Journal | Biochemistry |
| Year | 1998 |
| Volume | 37 |
| Pages | 1204-14 |
| Authors | Bersch B, Hernandez JF, Marion D, Arlaud GJ |
| Title |
Solution structure of the epidermal growth factor (EGF)-like module of human complement protease C1r, |
| Related PDB | 1apq |
| Related UniProtKB | P00736 |
| [8] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 9777414 |
| Journal | Immunobiology |
| Year | 1998 |
| Volume | 199 |
| Pages | 303-16 |
| Authors | Arlaud GJ, Rossi V, Thielens NM, Gaboriaud C, Bersch B, Hernandez JF |
| Title | Structural and functional studies on C1r and C1s: new insights into the mechanisms involved in C1 activity and assembly. |
| Related PDB | |
| Related UniProtKB | |
| [9] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 9777415 |
| Journal | Immunobiology |
| Year | 1998 |
| Volume | 199 |
| Pages | 317-26 |
| Authors | Gal P, Zavodszky P |
| Title | Structure and function of the serine-protease subcomponents of C1: protein engineering studies. |
| Related PDB | |
| Related UniProtKB | |
| [10] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 11414355 |
| Journal | Immunol Rev |
| Year | 2001 |
| Volume | 180 |
| Pages | 136-45 |
| Authors | Arlaud GJ, Gaboriaud C, Thielens NM, Rossi V, Bersch B, Hernandez JF, Fontecilla-Camps JC |
| Title | Structural biology of C1: dissection of a complex molecular machinery. |
| Related PDB | |
| Related UniProtKB | |
| [11] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 11445589 |
| Journal | J Biol Chem |
| Year | 2001 |
| Volume | 276 |
| Pages | 36233-40 |
| Authors | Lacroix M, Ebel C, Kardos J, Dobo J, Gal P, Zavodszky P, Arlaud GJ, Thielens NM |
| Title | Assembly and enzymatic properties of the catalytic domain of human complement protease C1r. |
| Related PDB | |
| Related UniProtKB | |
| [12] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 11823416 |
| Journal | EMBO J |
| Year | 2002 |
| Volume | 21 |
| Pages | 231-9 |
| Authors | Budayova-Spano M, Lacroix M, Thielens NM, Arlaud GJ, Fontecilla-Camps JC, Gaboriaud C |
| 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. |
| Related PDB | 1gpz |
| Related UniProtKB | |
| [13] | |
| Resource | |
| Comments | |
| Medline ID | |
| PubMed ID | 12429092 |
| Journal | Structure (Camb) |
| Year | 2002 |
| Volume | 10 |
| Pages | 1509-19 |
| Authors | Budayova-Spano M, Grabarse W, Thielens NM, Hillen H, Lacroix M, Schmidt M, Fontecilla-Camps JC, Arlaud GJ, Gaboriaud C |
| Title | Monomeric structures of the zymogen and active catalytic domain of complement protease c1r: further insights into the c1 activation mechanism. |
| Related PDB | 1md7 1md8 |
| Related UniProtKB | |
| Comments |
|---|
|
This enzyme belongs to the peptidase family-S1.
According to the literature [4], |
| Created | Updated |
|---|---|
| 2004-03-19 | 2012-08-08 |