Complement in human diseases: Lessons from complement deficiencies.

نویسندگان

  • Marina Botto
  • Michael Kirschfink
  • Paolo Macor
  • Matthew C Pickering
  • Reinhard Würzner
  • Francesco Tedesco
چکیده

Complement deficient cases reported in the second half of the last century have been of great help in defining the role of complement in host defence. Surveys of the deficient individuals have been instrumental in the recognition of the clinical consequences of the deficiencies. This review focuses on the analysis of the diseases associated with the deficiencies of the various components and regulators of the complement system and their therapeutic implications. The diagnostic approach leading to the identification of the deficiency is discussed here as a multistep process that starts with the screening assays and proceeds in specialized laboratories with the characterization of the defect at the molecular level. The organization of a registry of complement deficiencies is presented as a means to collect the cases identified in and outside Europe with the aim to promote joint projects on treatment and prevention of diseases associated with defective complement function.

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

منابع مشابه

Evaluation of Pasteurella multocida serotype B:2 resistance to immune serum and complement system

Members of gram-negative bacteria family Pasteurellaceae, include a large number of important economically human and veterinary pathogens. Organisms belonging to the family can colonize in mucosal surfaces of the respiratory, alimentary, genital tracts and cause diseases in various mammals, birds, and reptiles. Hemorrhagic septicemia is an acute disease of cattle and buffaloes in tropi...

متن کامل

Complement factors in Acute Myocardial Infarction and Unstable Angina

Background: Coronary artery disease (CAD) is one of the most important and lethal diseases in the world. CAD represents a board spectrum of disease from silent ischemia at one end to sudden cardiac death at the other end. The middle of this spectrum consists of acute myocardial infarction (AMI) and unstable angina pectoris (UA). Recent data show that the inflammatory process plays a major r...

متن کامل

Human genetic deficiencies reveal the roles of complement in the inflammatory network: lessons from nature.

Complement component C5 is crucial for experimental animal inflammatory tissue damage; however, its involvement in human inflammation is incompletely understood. The responses to gram-negative bacteria were here studied taking advantage of human genetic complement-deficiencies--nature's own knockouts--including a previously undescribed C5 defect. Such deficiencies provide a unique tool for inve...

متن کامل

Characterization of complement activity in serum of the four sturgeon species, Acipenser stellatus, A. baeri, A. nudiventris and Huso huso (Chondrostei: Acipenseridae)

Serum complement is valuable tool in determining the health status of fish. This study was conducted to examine in vitro activity of serum complement as an indicator of innate immunity in four sturgeon species, Acipenser stellatus, A. baeri, A. nudiventris and Huso huso (Chondrostei: Acipenseridae). The Effects of different temperatures, concentrations and volumes of serum on alternative comple...

متن کامل

ذخیره در منابع من


  با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید

برای دانلود متن کامل این مقاله و بیش از 32 میلیون مقاله دیگر ابتدا ثبت نام کنید

ثبت نام

اگر عضو سایت هستید لطفا وارد حساب کاربری خود شوید

عنوان ژورنال:
  • Molecular immunology

دوره 46 14  شماره 

صفحات  -

تاریخ انتشار 2009