Serpin | Alternative name(s) | Protease target or function | Involvement in disease |
---|---|---|---|
SERPINA1 | Antitrypsin | Extracellular; inhibition of neutrophil elastase | Deficiency results in emphysema: polymerization and retention in the ER results in cirrhosis [14,64,65] |
SERPINA2 | Antitrypsin-related protein | Not characterized, probable pseudogene | Â |
SERPINA3 | Antichymotrypsin | Extracellular; inhibition of cathepsin G | Deficiency results in emphysema (see [61] for a review) |
SERPINA4 | Kallistatin (PI4) | Extracellular, inhibition of kallikrein [68] | Â |
SERPINA5 | Protein C inhibitor (PAI-3) | Extracellular; inhibition of active protein C (see [69] for a review) | Angioedema |
SERPINA6 | Corticosteroid-binding globulin | Extracellular; non-inhibitory; cortisol binding | Deficiency linked to chronic fatigue [83,84] |
SERPINA7 | Thyroxine-binding globulin | Extracellular; non-inhibitory, thyroxine binding | Deficiency results in hypothyroidism [85] |
SERPINA8 | Angiotensinogen | Extracellular; non-inhibitory; amino-terminal cleavage by the protease renin results in release of the decapeptide angiotensin I | Certain variants linked to essential hypertension [86] |
SERPINA9 | Centerin | Extracellular; maintenance of naive B cells [70] | Â |
SERPINA10 | Protein Z-dependent proteinase inhibitor | Extracellular; inhibition of activated factor Z and XI | Deficiency linked to venous thromboembolic disease [87] |
SERPINA11 | XP_170754.3 | Not characterized | Â |
SERPINA12 | Vaspin | Extracellular; insulin-sensitizing adipocytokine [71] | Â |
SERPINA13 | XM_370772 | Not characterized | Â |
SERPINB1 | Monocyte neutrophil elastase inhibitor | Intracellular; inhibition of neutrophil elastase [72] | Â |
SERPINB2 | Plasminogen activator inhibitor-2 (PAI2) | Intracellular; inhibition of uPA (see [73] for a review) | Â |
SERPINB3 | Squamous cell carcinoma antigen-1 | Intracellular; cross-class inhibition of cathepsins L and V [6] | Â |
SERPINB4 | Squamous cell carcinoma antigen-2 | Intracellular; cross-class inhibition of cathepsin G and chymase [74] | Â |
SERPINB5 | Maspin | Intracellular; non-inhibitory; inhibition of metastasis through uncharacterized mechanism | Downregulation and/or intracellular location linked to tumor progression and overall prognosis [10] |
SERPINB6 | Proteinase inhibitor-6 (PI6) | Intracellular, inhibition of cathepsin G [75] | Â |
SERPINB7 | Megsin | Intracellular; megakaryocyte maturation [76] | IgA nephropathy |
SERPINB8 | Cytoplasmic antiproteinase 8 (PI8) | Intracellular; inhibition of furin [77] | Â |
SERPINB9 | Cytoplasmic antiproteinase 9 (PI9) | Intracellular, inhibition of granzyme B [78] | Â |
SERPINB10 | Bomapin (PI10) | Intracellular; inhibition of thrombin and trypsin [79] | Â |
SERPINB11 | Epipin | Intracellular | Â |
SERPINB12 | Yukopin | Intracellular; inhibition of trypsin [80] | Â |
SERPINB13 | Headpin (PI13) | Intracellular; inhibition of cathepsins L and K | Â |
SERPINC1 | Antithrombin | Extracellular; thrombin and factor Xa inhibitor | Deficiency results in thrombosis (see [88] for review) |
SERPIND1 | Heparin cofactor II | Extracellular; thrombin inhibitor | May contribute to thrombotic risk when combined with other deficiencies [89] |
SERPINE1 | Plasminogen activator inhibitor I (PAI1) | Extracellular; inhibitor of thrombin, uPA, tPA and plasmin | Abnormal bleeding [90] |
SERPINE2 | Protease nexin I (PI7) | Extracellular; inhibition of uPA and tPA | Â |
SERPINE3 | Hs.512272 | Not characterized | Â |
SERPINF1 | Pigment epithelium derived factor | Non-inhibitory; potent anti-angiogenic molecule [81] | Â |
SERPINF2 | Alpha-2-antiplasmin | Extracellular; plasmin inhibitor | Unrestrained fibrinolytic activity, bleeding [91] |
SERPING1 | C1 inhibitor | C1 esterase inhibitor | Angioedema [92] |
SERPINH1 | 47kDa heat-shock protein | Non-inhibitory molecular Chaperone for collagens [9] | Â |
SERPINI1 | Neuroserpin (PI12) | Extracellular; inhibitor of tPA, uPA and plasmin | Polymerization results in dementia [17] |
SERPINI2 | Myoepithelium-derived serine proteinase inhibitor (PI14) | Extracellular; inhibition of cancer metastasis [82] | Â |