KEGG   ENZYME: 3.4.21.42
Entry
EC 3.4.21.42                Enzyme                                 
Name
complement subcomponent C_overbar_1s_;
C1 esterase;
activated complement C1s;
complement C_overbar_1r_
Class
Hydrolases;
Acting on peptide bonds (peptidases);
Serine endopeptidases
Reaction(IUBMB)
Cleavage of Arg!Ala bond in complement component C4 to form C4a and C4b, and Lys(or Arg)!Lys bond in complement component C2 to form C2a and C2b: the "classical" pathway C3 convertase
Comment
Activated from proenzyme C1s in plasma by complement subcomponent C_overbar_1r_. In peptidase family S1 (trypsin family)
History
EC 3.4.21.42 created 1981
Orthology
K01331  complement component 1, s subcomponent
Genes
HSA716(C1S)
PTR451808(C1S)
PPS100991971(C1S)
GGO101145266(C1S)
PON100432027 100455685(C1S)
NLE100599399(C1S)
HMH116472620(C1S)
MCC714589(C1S)
MCF102134687 102137207(C1S)
MTHB126930476 126930486
MNI105484252(C1S)
CSAB103218499 103218857(C1S)
CATY105583054(C1S)
PANU101003889(C1S) 101009560
TGE112634416(C1S)
MLEU105541823(C1S)
RRO104664861 104680080(C1S)
RBB108542015
TFN117090593
PTEH111555895(C1S) 113222927 113222930 113222931
CANG105519191(C1S)
CJC100412274(C1S)
SBQ101034947(C1S)
CIMI108295012(C1S)
CSYR103252208(C1S)
MMUR105869013(C1S)
LCAT123640084(C1S)
PCOQ105805031(C1S)
OGA100952754(C1S)
MMU317677(C1s2) 50908(C1s1)
MPAH110314998 110316909(C1s)
RNO192262(C1s)
MCOC116099566(C1s)
ANU117715251(C1s)
MUN110548590(C1s)
CGE100759777(C1s)
MAUA101823163(C1s)
PROB127239131(C1s)
PLEU114708804(C1s)
MORG121443554(C1s)
MFOT126514859
AAMP119806528(C1s)
NGI103727488(C1s)
HGL101697979(C1s)
CPOC100722315(C1s)
CCAN109682778(C1s)
DORD105991022(C1s)
DSP122101967(C1s)
NCAR124983519
MMMA107154926(C1s)
OCU100348898
OPI101519319(C1S)
TUP102487452 102496444(C1S)
GVR103595776(C1S)
CFA486714(C1S)
CLUD112675376(C1S)
VVP112917318(C1S)
VLG121480300(C1S)
NPO129503430(C1S)
AML100472516(C1S)
UMR103656176(C1S)
UAH113257945(C1S)
UAR123781655(C1S)
ELK111159747
LLV125106220
MPUF101675967(C1S)
MNP132019897(C1S)
MLK131838481(C1S)
NVS122891831(C1S)
ORO101377797(C1S)
EJU114222555(C1S)
ZCA113922111(C1S)
MLX118025713(C1S)
NSU110581007(C1S)
LWW102736987(C1S)
FCA101088676(C1S)
PYU121022129(C1S)
PCOO112867394(C1S)
PBG122472396(C1S)
PVIV125170355(C1S)
LRUF124502523
PTG102948650(C1S)
PPAD109276089(C1S)
PUC125918798
AJU106965335
HHV120220413(C1S)
BTA767827(C1S)
BOM102285301(C1S)
BIU109558903(C1S)
BBUB102392362(C1S)
BBIS104992162(C1S)
CHX102189289(C1S)
OAS101107688(C1S)
BTAX128047669(C1S)
ODA120858755(C1S)
CCAD122423699(C1S)
MBEZ129536835(C1S)
SSC397274(C1S)
CFR102513921(C1S)
CBAI105075508(C1S)
CDK105098188(C1S)
VPC102534279(C1S)
BACU103003533(C1S)
BMUS118902480(C1S)
LVE103085588(C1S)
OOR101269409(C1S)
DLE111176708(C1S)
PCAD102987496(C1S)
PSIU116761113(C1S)
NASI112391771(C1S)
ECB100060261(C1S)
EPZ103563702(C1S)
EAI106847309(C1S)
MYB102255615(C1S)
MYD102755154(C1S)
MMYO118651039(C1S)
MLF102433220(C1S)
PKL118730585(C1S)
EFUS103289040(C1S)
MNA107544114(C1S)
DRO112321891(C1S)
SHON119002258(C1S)
AJM119065711(C1S)
PDIC114513721(C1S)
PHAS123810667(C1S)
MMF118624397(C1S)
PPAM129085046(C1S)
HAI109373658(C1S)
RFQ117029113(C1S)
PALE102897110(C1S)
PGIG120611678(C1S)
PVP105295936(C1S)
RAY107519836(C1S)
MJV108384826(C1S)
TOD119247759(C1S)
SARA101547063(C1S)
SETR126006663(C1S)
LAV100676140(C1S)
TMU101344169
ETF101663099(C1S)
DNM101436067(C1S)
MDO100016766(C1S)
GAS123249051(C1S)
SHR100917190(C1S)
AFZ127564067
PCW110213806(C1S)
OAA100085003(C1S)
GGA418294(C1S)
PCOC116239944(C1S)
MGP100549400(C1S)
CJO107318599(C1S)
TPAI128089839(C1S)
LMUT125685221(C1S)
NMEL110398269(C1S)
APLA101791779(C1S)
ACYG106047861(C1S)
CATA118247559(C1S)
AFUL116496502(C1S)
TGU100222051(C1S)
LSR110480852(C1S)
SCAN103812468(C1S)
PMOA120506494(C1S)
OTC121339352(C1S)
PRUF121351614(C1S)
GFR102043120(C1S)
FAB101809291(C1S)
OMA130259810(C1S)
PHI102112114(C1S)
PMAJ107207087(C1S)
CCAE111938155(C1S)
CCW104687135(C1S)
CBRC103613962(C1S)
ETL114058181(C1S)
ZAB102071401(C1S)
ZLE135453107(C1S)
ACHL103799611(C1S)
SVG106857861(C1S)
MMEA130574806(C1S)
HRT120748561(C1S)
FPG101915039(C1S)
FCH102049047(C1S)
CLV102093208(C1S)
EGZ104126544(C1S)
NNI104009512(C1S)
PCRI104026681(C1S)
PLET104616159(C1S)
EHS104513634(C1S)
PCAO104045746(C1S)
ACUN113479841(C1S)
TALA104362087(C1S)
PADL103920878(C1S)
AFOR103905459(C1S)
ACHC115343835(C1S)
HALD104312695(C1S)
HLE104831782(C1S)
AGEN126049063
GCL127029339
CCRI104157021(C1S)
CSTI104558311(C1S)
CMAC104475392(C1S)
MUI104540487(C1S)
BREG104631673(C1S)
FGA104078995(C1S)
GSTE104257359(C1S)
LDI104346353(C1S)
MNB103770372(C1S)
OHA104327130(C1S)
NNT104401342(C1S)
SHAB115601495(C1S)
PGUU104463167(C1S)
ACAR104520043(C1S)
CPEA104389443(C1S)
AVIT104279030(C1S)
CVF104292922(C1S)
RTD128902151(C1S)
CUCA104064359(C1S)
TEO104374572(C1S)
BRHI104495690(C1S)
AAM106497286(C1S)
AROW112973003(C1S)
NPD112945576(C1S)
TGT104565657(C1S)
DNE112991082(C1S)
SCAM104150723(C1S)
ASN102373441(C1S)
AMJ102572422(C1S)
CPOO109319374(C1S)
GGN109304790(C1S)
PSS102460272
CMY102946599(C1S)
CCAY125622817(C1S)
DCC119861421(C1S)
CPIC101944626(C1S)
TST117885631(C1S) 117886664
CABI116821039(C1S)
MRV120370719 120395337(C1S)
ACS100558615(c1s) 100560720
ASAO132767516
PVT110084650(C1S)
SUND121922922(C1S)
PBI103065043(C1S)
PMUR107285780(C1S)
CTIG120306115(C1S)
TSR106553465(C1S)
PGUT117661832(C1S)
APRI131193687(C1S)
PTEX113444439(C1S)
NSS113417163(C1S)
VKO123033203(C1S)
PMUA114587883(C1S)
PRAF128404589(C1S)
ZVI118097884(C1S)
HCG128342815(C1S)
GJA107120852 107123505
STOW125437225(C1S)
EMC129345422(C1S)
XLA733422(c1s.L)
XTR613055(c1s)
NPR108784394(C1S)
RTEM120946626(C1S)
BBUF121003772(C1S)
BGAR122941008(C1S)
MUO115457679(C1S)
GSH117350026(C1S)
SRX107714593 107724792
CAUA113081686 113099592
LROH127178407
OMC131522168
RKG130090827
TROS130557521
TDW130433448
MANU129448199
TRN134310857
AMEX103027368
EEE113574793
TRU101072918 115250374
TFS130532935(c1s.2)
TNGGSTEN00015325G001
NCC104941464
PGEO117461091
EMAC134861245
AFB129109514(c1s.2)
PSWI130213497(c1s.2)
MSAM119896349 119897430 119897431
MZE105940904
ONL100711586
OAU120442843(c1s.2)
CSAI133434870(c1s.2)
CVG107082194
ALIM106529166
LCF108880738(c1s.2)
BPEC110163037
OMY110503575
OKE118367891
SALP112074549
ELS105009774
PKI111835376
LOC102687802
PSPA121326273
ARUT117425904
PSEX120535739
LCM102354579(C1S)
CMK103189668 103190346
RTP109914345
CPLA122542919 122542923
HOC132836653
LERI129716458 129716463
 » show all
Reference
1  [PMID:6281620]
  Authors
Sim RB.
  Title
The human complement system serine proteases C1r and C1s and their proenzymes.
  Journal
Methods Enzymol 80 Pt C:26-42 (1981)
DOI:10.1016/s0076-6879(81)80006-7
Reference
2  [PMID:3500856]
  Authors
Mackinnon CM, Carter PE, Smyth SJ, Dunbar B, Fothergill JE
  Title
Molecular cloning of cDNA for human complement component C1s. The complete amino acid sequence.
  Journal
Eur J Biochem 169:547-53 (1987)
DOI:10.1111/j.1432-1033.1987.tb13644.x
  Sequence
[hsa:716]
Reference
3  [PMID:3052276]
  Authors
Muller-Eberhard HJ.
  Title
Molecular organization and function of the complement system.
  Journal
Annu Rev Biochem 57:321-47 (1988)
DOI:10.1146/annurev.bi.57.070188.001541
Reference
4  [PMID:2500154]
  Authors
Skoog MT, Mehdi S, Wiseman JS, Bey P.
  Title
The specificity of two proteinases that cleave adjacent to arginine, C1 esterase and acrosin, for peptide p-nitroanilide substrates.
  Journal
Biochim Biophys Acta 996:89-94 (1989)
DOI:10.1016/0167-4838(89)90099-X
Other DBs
ExplorEnz - The Enzyme Database: 3.4.21.42
IUBMB Enzyme Nomenclature: 3.4.21.42
ExPASy - ENZYME nomenclature database: 3.4.21.42
BRENDA, the Enzyme Database: 3.4.21.42
CAS: 80295-70-1
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