KEGG ENZYME: 3.4.21.93

ENZYME: 3.4.21.93

Entry

EC 3.4.21.93                Enzyme

Name

proprotein convertase 1;
prohormone convertase 3;
neuroendocrine convertase 1;
PC1

Class

Hydrolases;
Acting on peptide bonds (peptidases);
Serine endopeptidases

Reaction(IUBMB)

Release of protein hormones, neuropeptides and renin from their precursors, generally by hydrolysis of -Lys-Arg! bonds

Comment

A Ca2+-dependent enzyme, maximally active at about pH 5.5. Substrates include pro-opiomelanocortin, prorenin, proenkephalin, prodynorphin, prosomatostatin and proinsulin. Unlike prohormone convertase 2, does not hydrolyse proluteinizing-hormone-releasing-hormone. Unusually, processing of prodynorphin occurs at a bond in which P2 is Thr. Present in the regulated secretory pathway of neuroendocrine cells, commonly acting co-operatively with prohormone convertase 2. In peptidase family S8 (subtilisin family)

History

EC 3.4.21.93 created 1996

Orthology

K01359

proprotein convertase subtilisin/kexin type 1

Genes

HSA:	5122(PCSK1)

PTR:	461740(PCSK1)

PPS:	100988619(PCSK1)

GGO:	101129039(PCSK1)

PON:	100173253(PCSK1)

NLE:	100606392(PCSK1)

HMH:	116470985(PCSK1)

MCC:	699742(PCSK1)

MCF:	102142243(PCSK1)

MTHB:

126957464

MNI:	105484100(PCSK1)

CSAB:

103224951(PCSK1)

CATY:

105599043(PCSK1)

PANU:

101010467(PCSK1)

TGE:	112626110(PCSK1)

MLEU:

105546283(PCSK1)

RRO:	104654548(PCSK1)

RBB:	108520341(PCSK1)

TFN:	117064886(PCSK1)

PTEH:

111542718(PCSK1)

CANG:

105513427(PCSK1)

CJC:	100403507(PCSK1)

SBQ:	101042932(PCSK1)

CIMI:

108289441(PCSK1)

CSYR:

103254025(PCSK1)

MMUR:

105876203(PCSK1)

LCAT:

123648637(PCSK1)

PCOQ:

105805881(PCSK1)

OGA:	100957267(PCSK1)

MMU:	18548(Pcsk1)

MCAL:

110308002(Pcsk1)

MPAH:

110328930(Pcsk1)

RNO:	25204(Pcsk1)

MCOC:

116083517(Pcsk1)

ANU:	117700596(Pcsk1)

MUN:	110553616(Pcsk1)

CGE:	100757402(Pcsk1)

MAUA:

101841511(Pcsk1)

PROB:

127220884(Pcsk1)

PLEU:

114709982(Pcsk1)

MORG:

121455871(Pcsk1)

MFOT:

126488611

AAMP:

119809247(Pcsk1)

NGI:	103729900(Pcsk1)

HGL:	101704290(Pcsk1)

CPOC:

100732445(Pcsk1)

CCAN:

109684716(Pcsk1)

DORD:

105989808(Pcsk1)

DSP:	122117191(Pcsk1)

PLOP:

125340064(Pcsk1)

NCAR:

124986507

MMMA:

107135427(Pcsk1)

OCU:

100345424

OPI:	101528795(PCSK1)

TUP:	102471290(PCSK1)

GVR:	103587622(PCSK1)

CFA:	479149(PCSK1)

CLUD:

112653563(PCSK1)

VVP:	112915473(PCSK1)

VLG:	121490174(PCSK1)

NPO:	129501610(PCSK1)

AML:	100484463(PCSK1)

UMR:	103672232(PCSK1)

UAH:	113255069(PCSK1)

UAR:	123791580(PCSK1)

ELK:

111148364

LLV:

125100905

MPUF:

101677730(PCSK1)

MNP:	132028001(PCSK1)

MLK:	131832482(PCSK1)

NVS:	122917393(PCSK1)

ORO:	101371118(PCSK1)

EJU:	114200059(PCSK1)

ZCA:	113929954(PCSK1)

MLX:	118017027(PCSK1)

NSU:	110588836(PCSK1)

LWW:	102738182(PCSK1)

FCA:	101082627(PCSK1)

PYU:	121012781(PCSK1)

PCOO:

112860347(PCSK1)

PBG:	122491394(PCSK1)

PVIV:

125164483(PCSK1)

LRUF:

124506660

PTG:	102971097(PCSK1)

PPAD:

109254711(PCSK1)

PUC:

125934319

AJU:

106969006

HHV:	120236545(PCSK1)

BTA:	281967(PCSK1)

BOM:	102266289(PCSK1)

BIU:	109562342(PCSK1)

BBUB:

102409694(PCSK1)

BBIS:

104982533(PCSK1)

CHX:	102184429(PCSK1)

OAS:	443029(PCSK1)

BTAX:

128051163(PCSK1)

ODA:	120864422(PCSK1)

CCAD:

122439511(PCSK1)

MBEZ:

129558348(PCSK1)

SSC:	397103(PCSK1)

CFR:	102510598(PCSK1)

CBAI:

105064574(PCSK1)

CDK:	105100892(PCSK1)

VPC:	102539848(PCSK1)

BACU:

103011823(PCSK1)

BMUS:

118892980(PCSK1)

LVE:	103072078(PCSK1)

OOR:	101287435(PCSK1)

DLE:	111184190(PCSK1)

PCAD:

102980822(PCSK1)

PSIU:

116751401(PCSK1)

NASI:

112394752(PCSK1)

ECB:	100064886(PCSK1)

EPZ:	103560422(PCSK1)

EAI:	106847921(PCSK1)

MYB:	102259719(PCSK1)

MYD:	102763458(PCSK1)

MMYO:

118656885(PCSK1)

MLF:	102427061(PCSK1)

PKL:	118703440(PCSK1)

EFUS:

103288761(PCSK1)

MNA:	107539604(PCSK1)

DRO:	112307450(PCSK1)

SHON:

118977340(PCSK1)

AJM:	119055604(PCSK1)

PDIC:

114502355(PCSK1)

PHAS:

123824850(PCSK1)

MMF:	118641861(PCSK1)

PPAM:

129074691(PCSK1)

HAI:	109390165(PCSK1)

RFQ:	117025199(PCSK1)

PALE:

102890971(PCSK1)

PGIG:

120612559(PCSK1)

PVP:	105304316(PCSK1)

RAY:	107518284(PCSK1)

MJV:	108390924(PCSK1)

TOD:	119260977(PCSK1)

SARA:

101548146(PCSK1)

LAV:	100677414(PCSK1)

TMU:

101353481

ETF:	101651877(PCSK1)

DNM:	101438393(PCSK1)

MDO:	100010673(PCSK1)

GAS:	123231388(PCSK1)

SHR:	100925062(PCSK1)

AFZ:

127559906

PCW:	110223473(PCSK1)

OAA:	100081484(PCSK1)

GGA:	769230(PCSK1)

PCOC:

116236588(PCSK1)

MGP:	100547608(PCSK1)

CJO:	107306483(PCSK1)

TPAI:

128089788(PCSK1)

LMUT:

125686616(PCSK1)

NMEL:

110390176(PCSK1)

APLA:

101796157(PCSK1)

ACYG:

106048829(PCSK1)

CATA:

118258698(PCSK1)

AFUL:

116500462(PCSK1)

TGU:	100226064(PCSK1)

LSR:	110472368(PCSK1)

SCAN:

103823246(PCSK1)

PMOA:

120497551(PCSK1)

OTC:	121331992(PCSK1)

PRUF:

121352023(PCSK1)

GFR:	102042035(PCSK1)

FAB:	101816616(PCSK1)

OMA:	130265614(PCSK1)

PHI:	102111185(PCSK1)

PMAJ:

107198324(PCSK1)

CCAE:

111940930(PCSK1)

CCW:	104689825(PCSK1)

CBRC:

103616413(PCSK1)

ETL:	114057120(PCSK1)

ZAB:	102071185(PCSK1)

ACHL:

103803836(PCSK1)

SVG:	106857648(PCSK1)

MMEA:

130579758(PCSK1)

HRT:	120765664(PCSK1)

FPG:	101913861(PCSK1)

FCH:	102048239(PCSK1)

CLV:	102092106(PCSK1)

EGZ:	104122051(PCSK1)

NNI:	104015845(PCSK1) 104019230

PCRI:

104037707(PCSK1)

PLET:

104626526(PCSK1)

EHS:	104510645(PCSK1)

ACUN:

113490288(PCSK1)

TALA:

104364106(PCSK1)

PADL:

103918742(PCSK1)

AFOR:

103900083(PCSK1)

ACHC:

115337343(PCSK1)

HALD:

104321998(PCSK1)

HLE:	104832533(PCSK1)

AGEN:

126036342

GCL:

127027342

CCRI:

104167556(PCSK1)

CSTI:

104553579

CMAC:

104485983(PCSK1)

BREG:

104639357(PCSK1)

FGA:	104069084(PCSK1)

GSTE:

104263626(PCSK1)

LDI:	104349200(PCSK1)

MNB:

103771835

OHA:	104331739(PCSK1)

NNT:	104399501(PCSK1)

SHAB:

115601075(PCSK1)

DPUB:

104305612(PCSK1)

PGUU:

104461347(PCSK1)

ACAR:

104524047(PCSK1)

CPEA:

104394118(PCSK1)

AVIT:

104279224

CVF:	104285829(PCSK1)

RTD:	128903031(PCSK1)

CUCA:

104061096(PCSK1)

BRHI:

104496861(PCSK1)

AAM:	106485770 106488897(PCSK1)

AROW:

112974490(PCSK1)

NPD:	112951408(PCSK1)

TGT:	104566475(PCSK1) 104580040

DNE:	112980286(PCSK1)

SCAM:

104150384(PCSK1)

ASN:	102377633(PCSK1)

AMJ:	102564458(PCSK1)

CPOO:

109307699(PCSK1)

GGN:	109297219(PCSK1)

PSS:	102461055(PCSK1)

CMY:	102934087(PCSK1)

CCAY:

125636839(PCSK1)

DCC:	119856136(PCSK1)

CPIC:

101938233(PCSK1)

TST:	117879548(PCSK1)

CABI:

116835664(PCSK1)

MRV:	120408639(PCSK1)

ACS:	100565158(pcsk1)

ASAO:

132767163(PCSK1)

PVT:	110078772(PCSK1)

SUND:

121920967(PCSK1)

PBI:	103055161(PCSK1)

PMUR:

107302787(PCSK1)

CTIG:

120306718(PCSK1)

TSR:	106542108(PCSK1)

PGUT:

117679081(PCSK1)

APRI:

131192439(PCSK1)

PTEX:

113448531(PCSK1)

NSS:	113415254(PCSK1)

VKO:	123031580(PCSK1)

PMUA:

114606645(PCSK1)

PRAF:

128423369(PCSK1)

ZVI:	118076775(PCSK1)

HCG:	128346760(PCSK1)

GJA:	107119975(PCSK1)

STOW:

125436439(PCSK1)

EMC:	129334590(PCSK1)

XLA:	108707229(pcsk1.S)

XTR:	100496933(pcsk1)

NPR:	108790052(PCSK1)

MUO:	115462310(PCSK1)

GSH:	117352619(PCSK1)

DRE:	100005716(pcsk1)

SRX:	107739678 107754628

SANH:

107661910 107665244

SGH:	107584544(pcsk1) 107600928

CCAR:

109045223 109074034

CAUA:

113038837(pcsk1) 113063922

CGIB:

127941323 127986629

PTET:

122326897(pcsk1)

LROH:

127152493(pcsk1)

OMC:	131529376(pcsk1)

PPRM:

120490799(pcsk1)

RKG:	130090791(pcsk1)

MAMB:

125253063(pcsk1)

CIDE:

127504133 127525348

MASI:

127429212 127431248

TROS:

130570602(pcsk1)

TDW:	130408462(pcsk1)

MANU:

129424488(pcsk1)

IPU:

108279303

IFU:	128622601(pcsk1)

PHYP:

113535469(pcsk1)

SMEO:

124398413(pcsk1)

TFD:	113646394(pcsk1)

TVC:	132860272(pcsk1)

AMEX:

103035459(pcsk1)

CMAO:

118797500 118820256(pcsk1)

EEE:	113573749(pcsk1)

CHAR:

105902475(pcsk1)

TRU:	101067633(pcsk1)

TFS:	130524202(pcsk1) 130533158

TNG:	GSTEN00020152G001

LCO:	104923770 104928927(pcsk1)

NCC:	104957061(pcsk1)

TBEN:

117475445(pcsk1) 117480740

CGOB:

115008554(pcsk1) 115021661

PGEO:

117446970(pcsk1)

GACU:

117555251 117561867(pcsk1)

EMAC:

134862067 134882272(pcsk1)

ELY:	117257436(pcsk1) 117266197

EFO:	125887391(pcsk1) 125893281

PLEP:

121946024 121949891(pcsk1)

SLUC:

116034811(pcsk1) 116066554

ECRA:

117944094(pcsk1) 117945770

ESP:	116688116(pcsk1) 116690692

PFLV:

114554546(pcsk1) 114557388

GAT:	120810024 120834701(pcsk1)

PPUG:

119193893(pcsk1) 119197399

AFB:	129105992(pcsk1) 129109234

CLUM:

117727952 117731044(pcsk1)

PSWI:

130208301(pcsk1) 130213296

MSAM:

119886831(pcsk1) 119898035

SCHU:

122864174(pcsk1) 122882240

CUD:	121506991(pcsk1) 121513486

ALAT:

119005929 119021957(pcsk1)

MZE:	101467660(pcsk1)

ONL:	100534403(pcsk1)

OAU:	116326485(pcsk1)

OLA:	101161187 101174018(pcsk1)

OML:	112143846 112145446(pcsk1)

CSAI:

133440587 133448878(pcsk1)

XMA:	102231210(pcsk1) 106699876

XCO:	114135174(pcsk1) 114144237

XHE:	116710597(pcsk1) 116716835

PRET:

103464990(pcsk1)

PFOR:

103139591(pcsk1) 103151010

PLAI:

106949162(pcsk1) 106950884

PMEI:

106927188(pcsk1)

GAF:	122831727 122834414(pcsk1)

PPRL:

129358273(pcsk1) 129367733

CVG:	107088562 107091393(pcsk1)

CTUL:

119778565(pcsk1) 119795641

GMU:	124856155(pcsk1) 124865699

NFU:	107385468(pcsk1)

KMR:	108234188 108239887(pcsk1)

ALIM:

106523578(pcsk1) 106528261

NWH:	119410401 119412205(pcsk1)

AOCE:

111564532 111581198(pcsk1)

MCEP:

125007069(pcsk1) 125015863

CSEM:

103386425(pcsk1)

POV:	109628877(pcsk1) 109632018

SSEN:

122768375(pcsk1) 122774677

HHIP:

117761679(pcsk1) 117777394

HSP:	118105491(pcsk1) 118114289

SMAU:

118299900 118309975(pcsk1)

LCF:	108887327 108899706

SDU:	111221841(pcsk1)

SLAL:

111671003(pcsk1)

XGL:	120784663 120807348(pcsk1)

HCQ:	109522656(pcsk1)

SSCV:

125977840

SBIA:

133507997(pcsk1)

PEE:	133408535(pcsk1)

PTAO:

133484038(pcsk1)

BPEC:

110166707(pcsk1)

MALB:

109956208(pcsk1) 109964548

BSPL:

114843405 114855241(pcsk1)

SJO:	128356279(pcsk1) 128366865

SASA:

106565194 106581764 106583415

STRU:

115150203 115177405 115208259

OTW:	112233763 112254937(pcsk1)

OMY:	110493995 110504035 110528459(pcsk1)

OGO:	124003492 124027572(pcsk1)

ONE:	115114274 115143329 115143598

OKI:	109891631 109892371

OKE:	118379244(pcsk1) 118389026

SALP:

111962632 111970427

SNH:	120026237 120064634(pcsk1)

CCLU:

121543941 121575510 121578046(pcsk1)

ELS:	105008684 105016778(pcsk1)

SFM:	108928574(pcsk1) 108937256

PKI:	111843765(pcsk1)

AANG:

118213112(pcsk1) 118237356

LOC:	102685299(pcsk1) 107078452

PSPA:

121305806 121313229 121316242

ARUT:

117402762 117403513 117973454

PSEX:

120525368 120532903(pcsk1)

LCM:	102356617(PCSK1)

CMK:	103178388(pcsk1)

CPLA:

122550661 122559736(pcsk1)

HOC:	132816742 132825846(pcsk1)

LERI:

129695881(pcsk1) 129697375 129698179

PMRN:

116953638

LRJ:	133355061(PCSK4)

BFO:

118409753

BBEL:

109462268 109482637 109484828

SCLV:

120346276

LPIC:

129258750

APLC:

110983302 110988880

AJC:	117100115 117122554

ECOE:

129942790

HIS:

119658457

AME:

410438

ALAB:

122716064

ADR:

102675390

AFLR:

100871573

BIM:

100740253

BTER:

100648653

FVI:

122535693

CCAL:

108632970

OBB:

114872734

OLG:

117603559

SOC:

105194699

MPHA:

105832146

AEC:

105151284

ACEP:

105618631

PBAR:

105422178

VEM:

105562269

HST:

105186252

DQU:

106744203

CFO:

105256335

FEX:

115235749

LHU:

105675912

PGC:

109854544

OBO:

105287489

PCF:

106790738

PFUC:

122517846

VPS:

122637643

VCRB:

124423104

VVE:

124957899

NVI:

100115928

CSOL:

105365254

TPRE:

106660601

LHT:

122505100

LBD:

127285344

MDL:

103577201

CGLO:

123261175

FAS:

105270428

AGIF:

122854046

CINS:

118069353

VCAN:

122415965

CCIN:

107269531

DSM:

124413630

NPT:

124221884

NFB:

124185040

NLO:

107223046

NVG:

124307938

AROA:

105693339

TCA:

661661

DPA:

109543176

SOY:

115888807

AGRG:

126740932

ATD:

109609666

CSET:

123315146

AGB:

108915560

LDC:

111510090

NVL:	108559489 108564649

APLN:

108734297

PPYR:

116164260

OTU:

111413712

BMOR:

101741624

BMAN:

114240289

MSEX:

115448317

DPL:	KGM_215392

NIQ:

126773485

VCD:

124534771

MCIX:

123659254

PMAC:

106711372

PPOT:

106111529

PXU:

106119256

PRAP:

111003596

PBX:

123707344

PNAP:

125050633

ZCE:

119839762

CCRC:

123698679

LSIN:

126975494

HAW:

110376925

HZE:

124636801

TNL:

113503398

SLIU:

111355206

OFU:

114360672

PGW:

126370665

API:	100161992 100166094

DNX:

107168153

AGS:	114121160 114121161

RMD:	113556361 113557255

ACOO:

126846510 126846575

DVT:	126893713 126894017

BTAB:

109043119 109043175

DCI:	103505210 103512963 103513453

CLEC:

106667998

HHAL:

106683322

NLU:

120356274

HVI:

124365882

MQU:	128982096 128987228 129002668 129004034

PHU:	Phum_PHUM246240

FOC:

113206906

TPAL:

117645894

ZNE:

110838089

CSEC:

111870149

SGRE:

126281373

BROR:

134527325

IEL:

124166751

FCD:

110863089

DPX:	DAPPUDRAFT_43136 DAPPUDRAFT_59868

DMK:

116931038

DPZ:

124349263

PVM:

113829446

PJA:

122265272

PCHN:

125047912

PMOO:

119595243

HAME:

121855724

PCLA:

123762676

CQD:

128693866

PTRU:

123506832

ESN:

126983070

HAZT:

108664938

EAF:

111705271

LSM:

121118452

TCF:

131879452

PPOI:

119100268

DSV:

119449826

RSAN:

119382798

RMP:

119169333

TUT:

107362087

DPTE:

113791235

DFR:

124493883

CSCU:

111616523 111619886

PTEP:

107452277

ABRU:

129956177 129985133

UDV:	129225309 129232147

SDM:

118185696

LPOL:

106473941

PMEO:

129586529 129600864 129602351

HRO:	HELRODRAFT_193616 HELRODRAFT_74170

LGI:	LOTGIDRAFT_102960

PVUL:

126809519 126817679

PCAN:

112554321

BGT:

106078663

GAE:	121371772 121379630

HRF:	124114087 124115669

HRJ:	124254254 124257971

CRG:

105344671

CVN:	111103856 111128901

CANU:

128176473

OED:	125649115 125677735

MYI:

110462830

PMAX:

117334159

MCAF:

127734383

MMER:

123523001 123541536 123547153 123552686

RPHI:

132745223 132747204 132758737 132759675

DPOL:

127843859 127845818 127877737 127881056

OBI:	106874530 106875064

OSN:

115222333

LAK:	106155046 106160625 106173102

SMM:	Smp_149400

NVE:	5504528 5512606 5519937

EPA:	110239568 110248170 110248187 110251909

ATEN:

116288300 116290691 116290698 116301779 116304938

ADF:	107351431 107358341

AMIL:

114961986 114965472 114965473 114970251

PDAM:

113668359 113670666 113675011 113678927

SPIS:

111325458 111338335

DGT:	114521600 114522474 114537130 114537656 114537804 114540550

XEN:	124436810 124443035 124446506 124459460

HMG:	100201910 100202929 100205483

HSY:	130613855 130639822 130642775 130644785 130655201

TAD:	TRIADDRAFT_22925

» show all

Reference

1 [PMID:2169760]

Authors

Seidah NG, Gaspar L, Mion P, Marcinkiewicz M, Mbikay M, Chretien M.

Title

cDNA sequence of two distinct pituitary proteins homologous to Kex2 and furin gene products: tissue-specific mRNAs encoding candidates for pro-hormone processing proteinases.

Journal

DNA Cell Biol 9:415-24 (1990)
DOI:10.1089/dna.1990.9.415

Sequence

[mmu:18548]

Reference

2 [PMID:1988934]

Authors

Smeekens SP, Avruch AS, LaMendola J, Chan SJ, Steiner DF

Title

Identification of a cDNA encoding a second putative prohormone convertase related to PC2 in AtT20 cells and islets of Langerhans.

Journal

Proc Natl Acad Sci U S A 88:340-4 (1991)
DOI:10.1073/pnas.88.2.340

Sequence

[ag:AAA39896]

Reference

3 [PMID:1429684]

Authors

Steiner DF, Smeekens SP, Ohagi S, Chan SJ.

Title

The new enzymology of precursor processing endoproteases.

Journal

J Biol Chem 267:23435-8 (1992)

Reference

4 [PMID:7845206]

Authors

Seidah NG, Chretien M.

Title

Pro-protein convertases of subtilisin/kexin family.

Journal

Methods Enzymol 244:175-88 (1994)
DOI:10.1016/0076-6879(94)44015-8

Reference

5 [PMID:7741698]

Authors

Jean F, Basak A, DiMaio J, Seidah NG, Lazure C.

Title

An internally quenched fluorogenic substrate of prohormone convertase 1 and furin leads to a potent prohormone convertase inhibitor.

Journal

Biochem J 307 ( Pt 3):689-95 (1995)
DOI:10.1042/bj3070689

Other DBs

ExplorEnz - The Enzyme Database:

3.4.21.93

IUBMB Enzyme Nomenclature:

3.4.21.93

ExPASy - ENZYME nomenclature database:

3.4.21.93

BRENDA, the Enzyme Database:

3.4.21.93

CAS:	99676-46-7

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