Table 1.

Human serpins and pseudogenes, adapted from ref [42] and updated with data from ref [10]

Serpin	Synonyms	Size	RCL (P4-P4')	Chromosomal Location	Target Proteinases	Cofactors
CLADE A
SERPINA1	α1proteinase inhibitor (α1PI), α1antitrypsin (α1AT)	394 (418)	AIPM--SIPP	14q32.1	neutrophil elastase
SERPINA2	α1AT-related protein	399 (420)	EKAW--SKYQ	14q32.1
SERPINA3	α1antichymotrypsin (α1ACT)	398 (423)	ITLL--SALV	14q32.1	cathepsin G, chymase prostate specific antigen
SERPINA4	kallistatin, kallikrein inhibitor, PI4	401 (427)	IKFF--SAQT	14q32.1	tissue kallikrein
SERPINA5	protein C inhibitor (PCI), plasminogen activator inhibitor -3 (PAI-3)	387 (406)	FTFR--SARL	14q32.1	activated protein C, uPA. plasma kallikrein acrosin	heparin
SERPIN A6	corticosteroid binding globulin (CBG)	383 (405)	LNLT--SKPI	14q32.1	elastase substrate non-inhibitory	.
SERPINA7	thyroxin binding globulin	395 (415)	LSDQ--PENT	Xq22.2	non-inhibitory	.
SERPINA7P1				Xq22.3
SERPINA8	angiotensinogen (AGT)	452 (485)	NKPE--VLEV	1q42-q43	renin substrate non-inhibitory
SERPINA9	centerin	434	FIVR--SKDG	14q32.13
SERPINA10	protein Z-dependent protease inhibitor (ZPI)	423 (444)	ITAY--SMPP	14q32.13	factor Xa, factor XIa	protein Z, Ca2+, phospholipid , heparin
SERPINA11		pseudogene		14q32.13
SERPINA12	vaspin	395 (414)	TLPM--ETPL	14q32.13	kallikrein 7
SERPINA13P		pseudogene
SERPINA15P		pseudogene
CLADE B
SERPINB1	leukocyte elastase inhibitor (LEI), monocyte/neutrophil elastase inhibitor (MNEI), PI2, EI, ELANH2	379	ATFC--MLMP	6p25	neutrophil elastase, cathepsin G, proteinase 3, cathepsin L
SERPINB2	plasminogen activator inhibitor-2 (PAI-2), placental PAI,	415	MTGR--TGHG	18q21.3	uPA	.
SERPINB3	squamous cell carcinoma antigen 1 (SCCA1)	390	GFGS--SPAS	18q21.3	cathepsin K, cathepsin L cathepsin S
SERPINB4	squamous cell carcinoma antigen 2 (SCCA2), leupin	390	VVEL--SSPS	18q21.3	cathepsin G, chymase
SERPINB5	maspin, PI5	375	ILQH--KDEL	18q21.3	non-inhibitory
SERPINB6	placental thrombin inhibitor (PTI), PI6, cytoplasmic antiprotease (CAP)	376	MMMR--CARF	6p25	thrombin plasmin chymotrypsin cathepsin G
SERPINB7	megsin	380	IVEK--QLPQ	18q21.3
SERPINB8	cytoplasmic antiprotease 2 (CAP2), PI8	374	RNSR--CSRM	18q21.3	furin thrombin subtilisin A
SERPINB8P1		pseudogene		6p25
SERPINB9	cytoplasmic antiprotease 3 (CAP3), PI9	376	VVAE--CCME	6p25	granzyme B, caspase 1 (ICE), subtilisin A
SERPINB9P		pseudogene		6p25.2
SERPINB10	bomapin, PI10	397	IDIR--IRVP	18q21.3	thrombin
SERPINB11	epipin	392	IAVK--SLPM	18q21.3
SERPINB12	yukopin	405	VSER--SLRS	18q21.3	trypsin, plasmin
SERPINB13	headpin, hurpin, PI13	391	FTVT--SAPG	18q21.3
CLADE C
SERPINC1	antithrombin (AT), antithrombin III (ATIII)	432 (464)	IAGR--SLNP	1q23-q25	thrombin, factor Xa factor IXa	heparin, heparan sulfate
CLADE D
SERPIND1	heparin cofactor II (HCII), leuserpin 2	480 (499)	FMPL--STQV	22q11	thrombin	heparin, heparan sulfate, dermatan sulfate
CLADE E
SERPINE1	plasminogen activator inhibitor-1 (PAI-1), endothelial PAI	379 (402)	VSAR--MAPE	7q21.3-q22	uPA, tPA, thrombin, activated protein C	vitronectin
SERPINE2	protease nexin 1 (PN1), glia derived nexin (GDN)	379 (398)	LIAR--SSPP	2q33-q35	uPA, tPA, thrombin acrosin factor XIa	heparin
SERPINE3		404 (424)	LLKR--SRIP	13q14.3
CLADE F
SERPINF1	pigment epithelium-derived factor (PEDF), EPC-1	403 (418)	PAHL--TFPL	17p13.3	non-inhibitory
SERPINF2	α2antiplasmin (α2AP), A2AP	452 (491)	AMSR--MSLS	17pter-p12	plasmin
CLADE G
SERPING1	C1-inhibitor (C1 INH)	478 (500)	SVAR--TLLV	11q11-q13.1	C1r, C1s plasma kallikrein
CLADE H
SERPINH1	heat shock protein 47 (HSP47), collagen-binding protein 1 (CBP1), colligin 1	400 (417)	EELR--SPKL	11p13.5	non-inhibitory
SERPINH1P1				9p13.3
CLADE I
SERPINI1	neuroserpin	394 (410)	AISR--MAVL	3q26	tPA uPA plasmin
SERPINI2	myoepithelium-derived serine proteinase inhibitor, MEPI, pancpin	387 (405)	IPVI--MSLA	3q26