The Role of MASP-1/3 in Complement Activation

  • Hideharu Sekine
  • Minoru Takahashi
  • Daisuke Iwaki
  • Teizo FujitaEmail author
Part of the Advances in Experimental Medicine and Biology book series (AEMB, volume 735)


The complement system, which consists of more than 30 plasma and cell surface proteins, is activated by three pathways: the classical, lectin, and alternative pathways, leading to the generation of opsonins and pathogen destruction. In the lectin pathway, mannose-binding lectin (MBL) and ficolins act as pattern recognition molecules for pathogens, resulting in the activation of MBL-associated serine proteases (MASPs: MASP-1, MASP-2, and MASP-3). Among these proteases, MASP-2 is a key enzyme that cleaves C4 and C2 to assemble a C3 convertase (C4b2a). However, the physiological function of MASP-1 and MASP-3 remains unclear. To investigate the roles of MASP-1 and MASP-3, we generated a MASP-1- and MASP-3-deficient (M1/3 KO) mouse model and found that the deficient mice lacked alternative pathway activation because factor D (Df) remained as a proenzyme in the serum. MASP-1 and MASP-3 were able to convert the proenzyme of Df to an active form in vitro. In addition, MASP-1 was able to activate MASP-2 and MASP-3 as C1r activates C1s. Thus, MASP-1 and MASP-3 seem to be involved in activation of both the lectin and alternative pathways.


Alternative Pathway Lectin Pathway Carbohydrate Recognition Domain Alternative Pathway Activation Pattern Recognition Molecule 
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.


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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  • Hideharu Sekine
    • 1
  • Minoru Takahashi
    • 1
  • Daisuke Iwaki
    • 1
  • Teizo Fujita
    • 1
    • 2
    Email author
  1. 1.Department of ImmunologyFukushima Medical University School of MedicineFukushimaJapan
  2. 2.Fukushima Prefectural General Hygiene InstituteFukushimaJapan

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