Table 1.
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 |