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Springer Seminars in Immunopathology

, Volume 27, Issue 3, pp 299–319 | Cite as

Mannan-binding-lectin-associated serine proteases, characteristics and disease associations

  • Rikke Sørensen
  • Steffen ThielEmail author
  • Jens C. Jensenius
Original Article

Abstract

Mannan-binding lectin (MBL)-associated serine proteases (MASPs) circulate in plasma as zymogens in complexes with MBL and with L- and H-ficolin. Upon binding of MBL or ficolin to pathogen-associated molecular patterns, the MASPs are activated. MASP-2 can now cleave C4 and C2 to generate the C3 convertase, C4bC2b. The functions of the other two MASPs, MASP-1 and MASP-3 have not been elucidated. MASP-1 can cleave C2, and with low efficiency also C3, and may serve a function through direct C3 activation. No natural substrate for MASP-3 has been identified. MBL deficiency, occurring at a frequency of about 10%, is the most common congenital immunodeficiency and is associated with susceptibility to infections and autoimmune disorders. Inherited MASP-2 deficiency has been described as the result of a mutation causing the exchange of aspartic acid with a glycine at position 105, a position in the first domain, CUB1, involved in calcium binding. This mutation abolishes the binding to MBL and ficolins, and deprives MASP-2 of functional activity. The index case suffered from recurrent severe infections and autoimmune reactions. The gene frequency of the mutation among Caucasians is 3.6%. It is not found in Chinese, who present a different mutation also associated with MASP-2 deficiency.

Keywords

Carbohydrate Recognition Domain Serine Protease Domain Epidermal Growth Factor Domain Active Site Serine Residue CUB2 Fragment 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Notes

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Copyright information

© Springer-Verlag 2005

Authors and Affiliations

  • Rikke Sørensen
    • 1
  • Steffen Thiel
    • 1
  • Jens C. Jensenius
    • 1
  1. 1.Department of Medical Microbiology and Immunology, Wilhelm Meyers AlléUniversity of AarhusAarhusDenmark

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