MBL deficiency
Other namesMannose-binding lectin deficiency
Structure of the MBL2 protein.
SpecialtyImmunology

MBL deficiency or mannose-binding lectin deficiency is an illness that has an impact on immunity. Low levels of mannose-binding lectin, an immune system protein, are present in the blood of those who have this illness. It's unclear if this deficiency increases the risk of recurrent infections in those who are affected.[1]

Signs and symptoms

Specific infection susceptibility has been linked to low MBL levels.[2] This is especially true when it comes to chronic illnesses like cystic fibrosis, chemotherapy-induced neutropenia, and weakened immunity, which are all present in the neonatal period.[3] Moreover, septic shock and death have been connected to MBL deficiency.[4]

Causes

Mannose-binding lectin deficiency can result from mutations in the MBL2 gene.[1]

Mechanism

Mannose-binding lectin (MBL) is a soluble innate immune system pattern recognition molecule.[5] MBL activates the complement lectin pathway by binding to glycoconjugates on the surface of a variety of clinically significant bacteria, viruses, and fungi that contain mannose, fucose, or N-acetylglucosamine.[6][7] MBL stimulates leucocyte chemotaxis and activation as well as phagocytosis through complement activation.[8][9] Additionally, MBL may contribute to the resolution of lung inflammation by apoptosis cell clearance and proinflammatory cytokine secretion suppression.[10][11]

Epidemiology

Depending on the definition of deficiency, MBL deficiency affects 10–30% of individuals, making it one of the most prevalent immune defects.[12]

References

  1. ^ a b "Mannose-binding lectin deficiency: MedlinePlus Genetics". MedlinePlus. May 1, 2018. Retrieved January 22, 2024.
  2. ^ Haibrich, Michelle; Ben-Shoshan, Moshe; Mccusker, Christine (2012). "Mannose Binding Lectin Deficiency: More than Meets the Eye". Clinical Medicine Insights: Pediatrics. SAGE Publications. 6: 89–94. doi:10.4137/cmped.s9860. ISSN 1179-5565. PMC 3620812. PMID 23641170.
  3. ^ Eddie Ip, W. K.; Takahashi, Kazue; Alan Ezekowitz, R.; Stuart, Lynda M. (June 29, 2009). "Mannose-binding lectin and innate immunity". Immunological Reviews. Wiley. 230 (1): 9–21. doi:10.1111/j.1600-065x.2009.00789.x. ISSN 0105-2896. PMID 19594626. S2CID 44299051.
  4. ^ Dommett, R. M.; Klein, N; Turner, M. W. (2006). "Mannose-binding lectin in innate immunity: past, present and future". Tissue Antigens. Wiley. 68 (3): 193–209. doi:10.1111/j.1399-0039.2006.00649.x. ISSN 0001-2815.
  5. ^ Kilpatrick, D (September 19, 2002). "Mannan-binding lectin: clinical significance and applications". Biochimica et Biophysica Acta (BBA) - General Subjects. Elsevier BV. 1572 (2–3): 401–413. doi:10.1016/s0304-4165(02)00321-5. ISSN 0304-4165. PMID 12223282.
  6. ^ Neth, Olaf; Jack, Dominic L.; Dodds, Alister W.; Holzel, Helen; Klein, Nigel J.; Turner, Malcolm W. (2000). "Mannose-Binding Lectin Binds to a Range of Clinically Relevant Microorganisms and Promotes Complement Deposition". Infection and Immunity. American Society for Microbiology. 68 (2): 688–693. doi:10.1128/iai.68.2.688-693.2000. ISSN 0019-9567. PMC 97193. PMID 10639434.
  7. ^ Møller-Kristensen, Mette; Ip, W. K. Eddie; Shi, Lei; Gowda, Lakshmi D.; Hamblin, Michael R.; Thiel, Steffen; Jensenius, Jens Chr.; Ezekowitz, R. Alan B.; Takahashi, Kazue (February 1, 2006). "Deficiency of Mannose-Binding Lectin Greatly Increases Susceptibility to Postburn Infection with Pseudomonas aeruginosa". The Journal of Immunology. The American Association of Immunologists. 176 (3): 1769–1775. doi:10.4049/jimmunol.176.3.1769. ISSN 0022-1767. PMID 16424207.
  8. ^ Thiel, Steffen; Vorup-Jensen, Thomas; Stover, Cordula M.; Schwaeble, Wilhelm; Laursen, Steen B.; Poulsen, Knud; Willis, Anthony C.; Eggleton, Paul; Hansen, Søren; Holmskov, Uffe; Reid, Kenneth B. M.; Jensenius, Jens C. (1997). "A second serine protease associated with mannan-binding lectin that activates complement". Nature. Springer Science and Business Media LLC. 386 (6624): 506–510. Bibcode:1997Natur.386..506T. doi:10.1038/386506a0. ISSN 0028-0836. PMID 9087411.
  9. ^ Wallis, Russell (2007). "Interactions between mannose-binding lectin and MASPs during complement activation by the lectin pathway". Immunobiology. Elsevier BV. 212 (4–5): 289–299. doi:10.1016/j.imbio.2006.11.004. ISSN 0171-2985. PMID 17544814.
  10. ^ Hodge, Sandra; Matthews, Geoffrey; Dean, Melinda M.; Ahern, Jessica; Djukic, Michael; Hodge, Greg; Jersmann, Hubertus; Holmes, Mark; Reynolds, Paul N. (2010). "Therapeutic Role for Mannose-Binding Lectin in Cigarette Smoke–Induced Lung Inflammation?". American Journal of Respiratory Cell and Molecular Biology. American Thoracic Society. 42 (2): 235–242. doi:10.1165/rcmb.2008-0486oc. ISSN 1044-1549. PMID 19411612.
  11. ^ Wang, Mingyong; Chen, Yue; Zhang, Yani; Zhang, Liyun; Lu, Xiao; Chen, Zhengliang (March 7, 2011). "Mannan-binding lectin directly interacts with Toll-like receptor 4 and suppresses lipopolysaccharide-induced inflammatory cytokine secretion from THP-1 cells". Cellular & Molecular Immunology. Springer Science and Business Media LLC. 8 (3): 265–275. doi:10.1038/cmi.2011.1. ISSN 1672-7681. PMC 4012877. PMID 21383675.
  12. ^ Eisen, Damon P. (July 7, 2009). "Mannose-Binding Lectin Deficiency and Respiratory Tract Infection". Journal of Innate Immunity. S. Karger AG. 2 (2): 114–122. doi:10.1159/000228159. ISSN 1662-811X. PMID 20375630.

Further reading

External links

Classification
Lymphoid and complement disorders causing immunodeficiency
Category