Table 1 |
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Function and dysfunction of human serpins |
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| Serpin |
Alternative name(s) |
Protease target or function |
Involvement in disease |
|
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| 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] |
|
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Law et al. Genome Biology 2006 7:216 doi:10.1186/gb-2006-7-5-216 |
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