Review
BibTex RIS Cite

Kompleman Sistemi ve Hastalıkları

Year 2023, Volume: 7 Issue: 2, 103 - 111, 31.08.2023

Öner Özdemir

https://doi.org/10.29058/mjwbs.1250481

Abstract

Kompleman sistemi immün sistemin çok eskiden beri bilinen bir parçası olup, son yıllarda artan yeni
literatür verileri ışığında gözden geçirilmesine ihtiyaç vardır. İlk olarak, kompleman sistem biyolojisi
ve kompleman elementlerinin işlevlerinden bahsedilecektir. Bu sisteme ait bilinen 3 yolağın işleme
mekanizması ve bu sistemin regülatör proteinleri anlatılacaktır. Kompleman hastalıklarının tanımı,
toplumdaki sıklığı, nasıl klinikte tanınabileceklerinden bahsedildikten sonra, eskiden beri bilinen
ve yeni yeni öğrendiğimiz bazı kompleman hastalıklardan bahsedilecektir. Son olarak, kompleman
hastalıklarının laboratuvar testlerinden bahsedilecektir.

Keywords

İmmünyetmezlik kompleman AH50 CH50 properdin MBL

Supporting Institution

yok

Project Number

yok

Thanks

yok

References

  • 1. Kemper C, Pangburn MK, Fishelson Z. Complement nomenclature 2014. Mol Immunol 2014;61(2):56-58.
  • 2. Grumach AS, Kirschfink M. Are complement deficiencies really rare? Overview on prevalence, clinical importance and modern diagnostic approach. Mol Immunol 2014;61(2):110-117.
  • 3. Ling M, Murali M. Analysis of the complement system in the clinical immunology laboratory. Clin Lab Med 2019;39(4):579- 590.
  • 4. Thielens NM, Tedesco F, Bohlson SS, Gaboriaud C, Tenner AJ. C1q: A fresh look upon an old molecule. Mol Immunol 2017;89:73-83.
  • 5. Schröder-Braunstein J, Kirschfink M. Complement deficiencies and dysregulation: Pathophysiological consequences, modern analysis, and clinical management. Mol Immunol 2019;114:299- 311.
  • 6. Coss SL, Zhou D, Chua GT, Aziz RA, Hoffman RP, Wu YL, Ardoin SP, Atkinson JP, Yu CY. The complement system and human autoimmune diseases. J Autoimmun 2022:102979.
  • 7. Skattum L, van Deuren M, van der Poll T, Truedsson L. Complement deficiency states and associated infections. Mol Immunol 2011;48(14):1643-1655.
  • 8. Lung T, Risch L, Risch M, Sakem B, Würzner R, Nydegger U. The utility of complement assays in clinical immunology: A comprehensive review. J Autoimmun 2018;95:191-200.
  • 9. Mayilyan KR. Complement genetics, deficiencies, and disease associations. Protein Cell 2012;3(7):487-496.
  • 10. Mathern DR, Heeger PS. Molecules great and small: The complement system. Clin J Am Soc Nephrol 2015;10(9):1636- 1650.
  • 11. Ekdahl KN, Persson B, Mohlin C, Sandholm K, Skattum L, Nilsson B. Interpretation of serological complement biomarkers in disease. Front Immunol 2018;9:2237.
  • 12. Abolhassani H, Azizi G, Sharifi L, Yazdani R, Mohsenzadegan M, Delavari S, Sohani M, Shirmast P, Chavoshzadeh Z, Mahdaviani SA, Kalantari A, Tavakol M, Jabbari-Azad F, Ahanchian H, Momen T, Sherkat R, Sadeghi-Shabestari M, Aleyasin S, Esmaeilzadeh H, Al-Herz W, Bousfiha AA, Condino- Neto A, Seppänen M, Sullivan KE, Hammarström L, Modell V, Modell F, Quinn J, Orange JS, Aghamohammadi A. Global systematic review of primary immunodeficiency registries. Expert Rev Clin Immuno 2020;16(7):717-732.
  • 13. Frazer-Abel A, Kirschfink M, Prohászka Z. Expanding horizons in complement analysis and quality control. Front Immunol 2021;12:697313
  • 14. Keizer MP, Wouters D, Schlapbach LJ, Kuijpers TW. Restoration of MBL-deficiency: Redefining the safety, efficacy and viability of MBL-substitution therapy. Mol Immunol 2014;61(2):174-184.
  • 15. Urquhart J, Roberts R, de Silva D, Shalev S, Chervinsky E, Nampoothiri S, Sznajer Y, Revencu N, Gunasekera R, Suri M, Ellingford J, Williams S, Bhaskar S, Clayton-Smith J. Exploring the genetic basis of 3MC syndrome: Findings in 12 further families. Am J Med Genet A 2016;170A(5):1216-1224.
  • 16. Chen JY, Cortes C, Ferreira VP. Properdin: A multifaceted molecule involved in inflammation and diseases. Mol Immunol 2018;102:58-72.
  • 17. Józsi M, Zipfel PF. Factor H family proteins and human diseases. Trends Immunol 2008; 29(8):380-387.
  • 18. Zipfel PF, Mache C, Müller D, Licht C, Wigger M, Skerka C; European DEAP-HUS Study Group. DEAP-HUS: Deficiency of CFHR plasma proteins and autoantibody-positive form of hemolytic uremic syndrome. Pediatr Nephrol 2010;25(10):2009- 2019.
  • 19. Raina R, Vijayvargiya N, Khooblall A, Melachuri M, Deshpande S, Sharma D, Mathur K, Arora M, Sethi SK, Sandhu S. Pediatric atypical hemolytic uremic syndrome advances. Cells 2021;10(12):3580.
  • 20. Wong EKS, Kavanagh D. Diseases of complement dysregulation - an overview. Semin Immunopathol 2018;40(1):49-64.
  • 21. Ozen A, Comrie WA, Ardy RC, Domínguez Conde C, Dalgic B, Beser ÖF, Morawski AR, Karakoc-Aydiner E, Tutar E, Baris S, Ozcay F, Serwas NK, Zhang Y, Matthews HF, Pittaluga S, Folio LR, Unlusoy Aksu A, McElwee JJ, Krolo A, Kiykim A, Baris Z, Gulsan M, Ogulur I, Snapper SB, Houwen RHJ, Leavis HL, Ertem D, Kain R, Sari S, Erkan T, Su HC, Boztug K, Lenardo MJ. CD55 Deficiency, Early-Onset Protein-Losing Enteropathy, and Thrombosis. N Engl J Med 2017; 377(1):52-61.
  • 22. Ozen A, Kasap N, Vujkovic-Cvijin I, Apps R, Cheung F, Karakoc- Aydiner E, Akkelle B, Sari S, Tutar E, Ozcay F, Uygun DK, Islek A, Akgun G, Selcuk M, Sezer OB, Zhang Y, Kutluk G, Topal E, Sayar E, Celikel C, Houwen RHJ, Bingol A, Ogulur I, Eltan SB, Snow AL, Lake C, Fantoni G, Alba C, Sellers B, Chauvin SD, Dalgard CL, Harari O, Ni YG, Wang MD, Devalaraja- Narashimha K, Subramanian P, Ergelen R, Artan R, Guner SN, Dalgic B, Tsang J, Belkaid Y, Ertem D, Baris S, Lenardo MJ. Broadly effective metabolic and immune recovery with C5 inhibition in CHAPLE disease. Nat Immunol 2021;22(2):128- 139.
  • 23. Gialeli C, Gungor B, Blom AM. Novel potential inhibitors of complement system and their roles in complement regulation and beyond. Mol Immunol 2018;102:73-83.
  • 24. Lappegård KT, Bjerre A, Tjønnfjord GE, Mollnes TE. Therapeutic complement inhibition - from experimental to clinical medicine. Tidsskr Nor Laegeforen 2015;135(19):1745-1749.
  • 25. Prohászka Z, Nilsson B, Frazer-Abel A, Kirschfink M. Complement analysis 2016: Clinical indications, laboratory diagnostics and quality control. Immunobiology 2016; 221(11):1247-1258.
  • 26. Skattum L. Clinical Complement Analysis-An Overview. Transfus Med Rev 2019; 33(4):207-216.
  • 27. Brandwijk RJMGE, Michels MAHM, van Rossum M, de Nooijer AH, Nilsson PH, de Bruin WCC, Toonen EJM. Pitfalls in complement analysis: A systematic literature review of assessing complement activation. Front Immunol 2022;13:1007102.
  • 28. Ricklin D, Barratt-Due A, Mollnes TE. Complement in clinical medicine: Clinical trials, case reports and therapy monitoring. Mol Immunol 2017;89:10-21.
  • 29. Botto M, Kirschfink M, Macor P, Pickering MC, Würzner R, Tedesco F. Complement in human diseases: Lessons from complement deficiencies. Mol Immunol 2009;46(14):2774- 2783.
  • 30. López-Lera A, Corvillo F, Nozal P, Regueiro JR, Sánchez- Corral P, López-Trascasa M. Complement as a diagnostic tool in immunopathology. Semin Cell Dev Biol 2019;85:86-97.

Complement System and Disorders

Year 2023, Volume: 7 Issue: 2, 103 - 111, 31.08.2023

Öner Özdemir

https://doi.org/10.29058/mjwbs.1250481

Abstract

The complement system is a part of the immune system that has been known for a long time, and
it needs to be reviewed in the light of new literature data that has increased in recent years. Firstly,
complement system biology and functions of complement elements will be discussed. The mechanism
of the three known pathways of this system and the regulatory proteins of this system will be explained.
After defining the complement disorders, their frequency in the community, and how we recognize in the
clinic setting, some of the complement diseases that have been known for a long time and the diseases
have just learned will be mentioned. Finally, we will discuss laboratory tests of complement diseases.

Keywords

Immunodeficiency complement AH50 CH50 properdin MBL

Project Number

yok

References

  • 1. Kemper C, Pangburn MK, Fishelson Z. Complement nomenclature 2014. Mol Immunol 2014;61(2):56-58.
  • 2. Grumach AS, Kirschfink M. Are complement deficiencies really rare? Overview on prevalence, clinical importance and modern diagnostic approach. Mol Immunol 2014;61(2):110-117.
  • 3. Ling M, Murali M. Analysis of the complement system in the clinical immunology laboratory. Clin Lab Med 2019;39(4):579- 590.
  • 4. Thielens NM, Tedesco F, Bohlson SS, Gaboriaud C, Tenner AJ. C1q: A fresh look upon an old molecule. Mol Immunol 2017;89:73-83.
  • 5. Schröder-Braunstein J, Kirschfink M. Complement deficiencies and dysregulation: Pathophysiological consequences, modern analysis, and clinical management. Mol Immunol 2019;114:299- 311.
  • 6. Coss SL, Zhou D, Chua GT, Aziz RA, Hoffman RP, Wu YL, Ardoin SP, Atkinson JP, Yu CY. The complement system and human autoimmune diseases. J Autoimmun 2022:102979.
  • 7. Skattum L, van Deuren M, van der Poll T, Truedsson L. Complement deficiency states and associated infections. Mol Immunol 2011;48(14):1643-1655.
  • 8. Lung T, Risch L, Risch M, Sakem B, Würzner R, Nydegger U. The utility of complement assays in clinical immunology: A comprehensive review. J Autoimmun 2018;95:191-200.
  • 9. Mayilyan KR. Complement genetics, deficiencies, and disease associations. Protein Cell 2012;3(7):487-496.
  • 10. Mathern DR, Heeger PS. Molecules great and small: The complement system. Clin J Am Soc Nephrol 2015;10(9):1636- 1650.
  • 11. Ekdahl KN, Persson B, Mohlin C, Sandholm K, Skattum L, Nilsson B. Interpretation of serological complement biomarkers in disease. Front Immunol 2018;9:2237.
  • 12. Abolhassani H, Azizi G, Sharifi L, Yazdani R, Mohsenzadegan M, Delavari S, Sohani M, Shirmast P, Chavoshzadeh Z, Mahdaviani SA, Kalantari A, Tavakol M, Jabbari-Azad F, Ahanchian H, Momen T, Sherkat R, Sadeghi-Shabestari M, Aleyasin S, Esmaeilzadeh H, Al-Herz W, Bousfiha AA, Condino- Neto A, Seppänen M, Sullivan KE, Hammarström L, Modell V, Modell F, Quinn J, Orange JS, Aghamohammadi A. Global systematic review of primary immunodeficiency registries. Expert Rev Clin Immuno 2020;16(7):717-732.
  • 13. Frazer-Abel A, Kirschfink M, Prohászka Z. Expanding horizons in complement analysis and quality control. Front Immunol 2021;12:697313
  • 14. Keizer MP, Wouters D, Schlapbach LJ, Kuijpers TW. Restoration of MBL-deficiency: Redefining the safety, efficacy and viability of MBL-substitution therapy. Mol Immunol 2014;61(2):174-184.
  • 15. Urquhart J, Roberts R, de Silva D, Shalev S, Chervinsky E, Nampoothiri S, Sznajer Y, Revencu N, Gunasekera R, Suri M, Ellingford J, Williams S, Bhaskar S, Clayton-Smith J. Exploring the genetic basis of 3MC syndrome: Findings in 12 further families. Am J Med Genet A 2016;170A(5):1216-1224.
  • 16. Chen JY, Cortes C, Ferreira VP. Properdin: A multifaceted molecule involved in inflammation and diseases. Mol Immunol 2018;102:58-72.
  • 17. Józsi M, Zipfel PF. Factor H family proteins and human diseases. Trends Immunol 2008; 29(8):380-387.
  • 18. Zipfel PF, Mache C, Müller D, Licht C, Wigger M, Skerka C; European DEAP-HUS Study Group. DEAP-HUS: Deficiency of CFHR plasma proteins and autoantibody-positive form of hemolytic uremic syndrome. Pediatr Nephrol 2010;25(10):2009- 2019.
  • 19. Raina R, Vijayvargiya N, Khooblall A, Melachuri M, Deshpande S, Sharma D, Mathur K, Arora M, Sethi SK, Sandhu S. Pediatric atypical hemolytic uremic syndrome advances. Cells 2021;10(12):3580.
  • 20. Wong EKS, Kavanagh D. Diseases of complement dysregulation - an overview. Semin Immunopathol 2018;40(1):49-64.
  • 21. Ozen A, Comrie WA, Ardy RC, Domínguez Conde C, Dalgic B, Beser ÖF, Morawski AR, Karakoc-Aydiner E, Tutar E, Baris S, Ozcay F, Serwas NK, Zhang Y, Matthews HF, Pittaluga S, Folio LR, Unlusoy Aksu A, McElwee JJ, Krolo A, Kiykim A, Baris Z, Gulsan M, Ogulur I, Snapper SB, Houwen RHJ, Leavis HL, Ertem D, Kain R, Sari S, Erkan T, Su HC, Boztug K, Lenardo MJ. CD55 Deficiency, Early-Onset Protein-Losing Enteropathy, and Thrombosis. N Engl J Med 2017; 377(1):52-61.
  • 22. Ozen A, Kasap N, Vujkovic-Cvijin I, Apps R, Cheung F, Karakoc- Aydiner E, Akkelle B, Sari S, Tutar E, Ozcay F, Uygun DK, Islek A, Akgun G, Selcuk M, Sezer OB, Zhang Y, Kutluk G, Topal E, Sayar E, Celikel C, Houwen RHJ, Bingol A, Ogulur I, Eltan SB, Snow AL, Lake C, Fantoni G, Alba C, Sellers B, Chauvin SD, Dalgard CL, Harari O, Ni YG, Wang MD, Devalaraja- Narashimha K, Subramanian P, Ergelen R, Artan R, Guner SN, Dalgic B, Tsang J, Belkaid Y, Ertem D, Baris S, Lenardo MJ. Broadly effective metabolic and immune recovery with C5 inhibition in CHAPLE disease. Nat Immunol 2021;22(2):128- 139.
  • 23. Gialeli C, Gungor B, Blom AM. Novel potential inhibitors of complement system and their roles in complement regulation and beyond. Mol Immunol 2018;102:73-83.
  • 24. Lappegård KT, Bjerre A, Tjønnfjord GE, Mollnes TE. Therapeutic complement inhibition - from experimental to clinical medicine. Tidsskr Nor Laegeforen 2015;135(19):1745-1749.
  • 25. Prohászka Z, Nilsson B, Frazer-Abel A, Kirschfink M. Complement analysis 2016: Clinical indications, laboratory diagnostics and quality control. Immunobiology 2016; 221(11):1247-1258.
  • 26. Skattum L. Clinical Complement Analysis-An Overview. Transfus Med Rev 2019; 33(4):207-216.
  • 27. Brandwijk RJMGE, Michels MAHM, van Rossum M, de Nooijer AH, Nilsson PH, de Bruin WCC, Toonen EJM. Pitfalls in complement analysis: A systematic literature review of assessing complement activation. Front Immunol 2022;13:1007102.
  • 28. Ricklin D, Barratt-Due A, Mollnes TE. Complement in clinical medicine: Clinical trials, case reports and therapy monitoring. Mol Immunol 2017;89:10-21.
  • 29. Botto M, Kirschfink M, Macor P, Pickering MC, Würzner R, Tedesco F. Complement in human diseases: Lessons from complement deficiencies. Mol Immunol 2009;46(14):2774- 2783.
  • 30. López-Lera A, Corvillo F, Nozal P, Regueiro JR, Sánchez- Corral P, López-Trascasa M. Complement as a diagnostic tool in immunopathology. Semin Cell Dev Biol 2019;85:86-97.
There are 30 citations in total.

Details

Primary Language Turkish
Subjects Health Care Administration
Journal Section Review
Authors

Öner Özdemir 0000-0002-5338-9561

Project Number yok
Publication Date August 31, 2023
Acceptance Date July 5, 2023
Published in Issue Year 2023 Volume: 7 Issue: 2

Cite

Vancouver Özdemir Ö. Kompleman Sistemi ve Hastalıkları. Med J West Black Sea. 2023;7(2):103-11.
 Download Cover Image

Medical Journal of Western Black Sea is a scientific publication of Zonguldak Bulent Ecevit University Faculty of Medicine.

This is a refereed journal, which aims at achieving free knowledge to the national and international organizations and individuals related to medical sciences in publishedand electronic forms.

This journal is published three annually in April, August and December.
The publication language of the journal is Turkish and English.