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  Domain Name: PAI-1_nexin-1
Plasminogen activator inhibitor-1_like. Plasminogen activator inhibitor-1 (PAI-1) is the primary, fast-acting inhibitor of plasminogen activators. It is often bound to vitronectin, an abundant component of the extracellular matrix in many tissues. Protease nexin-1 is a potent serpin able to inhibit thrombin, plasmin, and plasminogen activators. PAI-1 and nexin-1 are members of the serpin superfamily and represent clade E. In general, SERine Proteinase INhibitors (serpins) exhibit conformational polymorphism shifting from native to cleaved, latent, delta, or polymorphic forms. Many serpins, such as antitrypsin and antichymotrypsin, function as serine protease inhibitors which regulate blood coagulation cascades. Non-inhibitory serpins perform many diverse functions such as chaperoning proteins or transporting hormones.
No pairwise interactions are available for this conserved domain.

Total Mutations Found: 74
Total Disease Mutations Found: 26
This domain occurred 31 times on human genes (62 proteins).



  ALPHA-2-PLASMIN INHIBITOR DEFICIENCY
  ANGIOEDEMA, HEREDITARY, TYPE I
  ANGIOEDEMA, HEREDITARY, TYPE II
  ANTICHYMOTRYPSIN BOCHUM 1
  ANTICHYMOTRYPSIN BONN 1
  ANTICHYMOTRYPSIN ISEHARA 1
  C1 INHIBITOR
  COMPLEMENT COMPONENT 4, PARTIAL DEFICIENCY OF, DUE TO DYSFUNCTIONAL
  DEAFNESS, AUTOSOMAL RECESSIVE 91
  ENCEPHALOPATHY, FAMILIAL, WITH NEUROSERPIN INCLUSION BODIES
  HYPERTENSION, ESSENTIAL, SUSCEPTIBILITY TO
  IGA NEPHROPATHY, PROGRESSION TO
  OSTEOGENESIS IMPERFECTA, TYPE VI
  OSTEOGENESIS IMPERFECTA, TYPE X
  PALMOPLANTAR KERATODERMA, NAGASHIMA TYPE
  PREECLAMPSIA, SUSCEPTIBILITY TO, INCLUDED;;
  RENAL TUBULAR DYSGENESIS
  VENOUS THROMBOSIS, SUSCEPTIBILITY TO


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Range on the Protein:  

   Protein ID            Protein Position

Domain Position:  


Feature Name:Total Found:
reactive center loop



















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Please Cite: Peterson, T.A., Adadey, A., Santana-Cruz ,I., Sun, Y., Winder A, Kann, M.G., (2010) DMDM: Domain Mapping of Disease Mutations. Bioinformatics 26 (19), 2458-2459.

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