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Mannan-binding lectin-associated serine protease-2 (MASP-2) deficiency in two patients with pulmonary tuberculosis and one healthy control

Cellular & Molecular Immunology volume 12, pages 119–121 (2015)Cite this article

Hereditary complement deficiencies are usually associated with increased susceptibility to infections and/or autoimmune diseases. Generally, they are rare: the majority have been detected in no more than a few dozen individuals. However, due to lack of population-based studies, such cases are usually detected by chance, e.g., in severely ill persons and their family members. An exception is mannan-binding lectin (MBL) deficiency, affecting perhaps 5%–10% of the population. MBL, like collectin-10, -11 and the ficolins (M-, L-, H-), is a pattern-recognition molecule, cooperating with MBL-associated serine proteases (MASPs) in the initiation of complement activation via the lectin pathway. In contrast, deficiency of other factors of the lectin pathway seems to be very rare.

Within the MASP family, three proteases (MASP-1, MASP-2, MASP-3) and two related, non-enzymatic proteins, MAp19 (sMAP) and MAp44 (MAP-1), have been identified. MASP-1, MASP-3 and MAp44 are the products of alternative splicing of the MASP1/3 gene while MASP-2 and MAp19 are synthesized under the control of the MASP2 gene. MASP-2 is responsible for activation of complement factors C4 and C2. MASP-2 is composed of six domains: CUB1, EGF, CUB2, CCP1, CCP2 and the serine protease domain.1

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References

  1. Thiel S, Steffensen R, Christensen IJ, Ip WK, Lau YL, Reason IJ et al. Deficiency of mannan-binding lectin associated serine protease-2 due to missense polymorphisms. Genes Immun 2007; 8: 154–163.

    Article  CAS  Google Scholar 

  2. Stengaard-Pedersen K, Thiel S, Gadjeva M, Moller-Kristensen M, Sorensen R, Jensen LT et al. Inherited deficiency of mannan-binding lectin associated serine protease 2. N Engl J Med 2003; 349: 554–560.

    Article  CAS  Google Scholar 

  3. Cedzynski M, Szemraj J, Swierzko AS, Bak-Romaniszyn L, Banasik M, Zeman K et al. Mannan-binding lectin insufficiency in children with recurrent infections of the respiratory system. Clin Exp Immunol 2004; 136: 304–311.

    Article  CAS  Google Scholar 

  4. Garcia-Laorden MI, Garcia-Saavedra A, de Castro FR, Violan JS, Rajas O, Blanquer J et al. Low clinical penetrance of mannose-binding lectin-associated serine protease 2 deficiency. J Allergy Clin Immunol 2006; 118: 1383–1386.

    Article  CAS  Google Scholar 

  5. Garcia-Laorden MI, Sole-Violan J, Rodriguez de Castro F, Aspa J, Briones ML, Garcia-Saavedra A et al. Mannose-binding lectin and mannose-binding lectin-associated serine protease 2 in susceptibility, severity, and outcome of pneumonia in adults. J Allergy Clin Immunol 2008; 122: 368–374.

    Article  CAS  Google Scholar 

  6. Stover C, Barrett S, Lynch NJ, Barker JN, Burden D, Trembath R et al. Functional MASP2 single nucleotide polymorphism plays no role in psoriasis. Br J Dermatol 2005; 152: 1313–1315.

    Article  CAS  Google Scholar 

  7. Olszowski T, Poziomkowska-Gęsicka I, Jensenius JC, Adler G . Lectin pathway of complement activation in a Polish woman with MASP-2 deficiency. Immunobiology 2013; in press (e-pub: 2013 Nov. 5th).

  8. Cedzynski M, Atkinson AP, Swierzko AS, MacDonald SL, Szala A, Zeman K et al. L-ficolin (ficolin-2) insufficiency is associated with combined allergic and infectious respiratory disease in children. Mol Immunol 2009; 47: 415–419.

    Article  CAS  Google Scholar 

  9. Olesen HV, Jensenius JC, Steffensen R, Thiel S, Schiotz PO . The mannan-binding lectin pathway and lung disease in cystic fibrosis—disfunction of mannan-binding lectin-associated serine protease 2 (MASP-2) may be a major modifier. Clin Immunol 2006; 121: 324–331.

    Article  CAS  Google Scholar 

  10. Segat L, Fabris A, Padovan L, Milanese M, Pirulli D, Lupo F et al. MBL2 and MASP2 gene polymorphisms in patients with hepatocellular carcinoma. J Viral Hepat 2008; 15: 387–391.

    Article  CAS  Google Scholar 

  11. Ytting H, Christensen IJ, Steffensen R, Alsner J, Thiel S, Jensenius JC et al. Mannan-binding lectin (MBL) and MBL-associated serine protease 2 (MASP-2) genotypes in colorectal cancer. Scand J Immunol 2011; 73: 122–127.

    Article  CAS  Google Scholar 

Download references

Acknowledgements

This work was supported by the European Union within European Regional Development Fund, through grant Innovative Economy (POIG. 01.01.02-10-107/09). We thank Professor Jens C Jensenius (University of Aarhus, Denmark) for providing anti-MASP-2 monoclonal antibodies and Dr D C Kilpatrick (Edinburgh, Scotland, UK) for critical reading of the manuscript and editorial suggestions.

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Author notes

  1. Dr A Swierzko, Laboratory of Immunobiology of Infections, Institute of Medical Biology, Polish Academy of Sciences, Lodowa 106, 93-232 Lodz, Poland. E-mail: aswierzko@cbm.pan.pl

Authors and Affiliations

  1. Laboratory of Immunobiology of Infections, Institute of Medical Biology, Polish Academy of Sciences, Lodz, Poland

    Anna Sokolowska, Agnieszka Szala, Anna St Swierzko & Maciej Cedzynski

  2. Institute of Tuberculosis and Lung Diseases, Warsaw, Poland

    Monika Kozinska & Ewa Augustynowicz-Kopec

  3. The Voivodeship Hospital of Lung Diseases in Jaroszowiec, Jaroszowiec, Poland

    Tomasz Niemiec

  4. Masovian Center of Lung Diseases and Tuberculosis Treatment, Otwock, Poland

    Maria Blachnio

  5. Laboratory of Mycobacterium Genetics and Physiology, Institute of Medical Biology, Polish Academy of Sciences, Lodz, Poland

    Jaroslaw Dziadek

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  1. Anna Sokolowska
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Sokolowska, A., Szala, A., St Swierzko, A. et al. Mannan-binding lectin-associated serine protease-2 (MASP-2) deficiency in two patients with pulmonary tuberculosis and one healthy control. Cell Mol Immunol 12, 119–121 (2015). https://doi.org/10.1038/cmi.2014.19

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