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Table 1 Function and dysfunction of human serpins

From: An overview of the serpin superfamily

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  
SERPINA3 Antichymotrypsin Extracellular; inhibition of cathepsin G Deficiency results in emphysema (see [61] for a review)
SERPINA4 Kallistatin (PI4) Extracellular, inhibition of kallikrein [68]  
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]  
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  
SERPINA12 Vaspin Extracellular; insulin-sensitizing adipocytokine [71]  
SERPINA13 XM_370772 Not characterized  
SERPINB1 Monocyte neutrophil elastase inhibitor Intracellular; inhibition of neutrophil elastase [72]  
SERPINB2 Plasminogen activator inhibitor-2 (PAI2) Intracellular; inhibition of uPA (see [73] for a review)  
SERPINB3 Squamous cell carcinoma antigen-1 Intracellular; cross-class inhibition of cathepsins L and V [6]  
SERPINB4 Squamous cell carcinoma antigen-2 Intracellular; cross-class inhibition of cathepsin G and chymase [74]  
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]  
SERPINB7 Megsin Intracellular; megakaryocyte maturation [76] IgA nephropathy
SERPINB8 Cytoplasmic antiproteinase 8 (PI8) Intracellular; inhibition of furin [77]  
SERPINB9 Cytoplasmic antiproteinase 9 (PI9) Intracellular, inhibition of granzyme B [78]  
SERPINB10 Bomapin (PI10) Intracellular; inhibition of thrombin and trypsin [79]  
SERPINB11 Epipin Intracellular  
SERPINB12 Yukopin Intracellular; inhibition of trypsin [80]  
SERPINB13 Headpin (PI13) Intracellular; inhibition of cathepsins L and K  
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  
SERPINE3 Hs.512272 Not characterized  
SERPINF1 Pigment epithelium derived factor Non-inhibitory; potent anti-angiogenic molecule [81]  
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]  
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]