Molecular Biology for Genetic Deficiencies of Complement Components in Rabbits

Complement in human diseases: Lessons from complement deficiencies.

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 re...

Complete complement components C4A and C4B deficiencies in human kidney diseases and systemic lupus erythematosus.

Although a heterozygous deficiency of either complement component C4A or C4B is common, and each has a frequency of approximately 20% in a Caucasian population, complete deficiencies of both C4A and C4B proteins are extremely rare. In this paper the clinical courses for seven complete C4 deficiency patients are described in detail, and the molecular defects for complete C4 deficiencies are eluc...

Inherited Classical and Alternative Pathway Complement Deficiencies in Children: A Single Center Experience

Systemic Lupus Erythematosus and Deficiencies of Early Components of the Complement Classical Pathway

The complement system plays an important role in the innate and acquired immune response against pathogens. It consists of more than 30 proteins found in soluble form or attached to cell membranes. Most complement proteins circulate in inactive forms and can be sequentially activated by the classical, alternative, or lectin pathways. Biological functions, such as opsonization, removal of apopto...

The Emerging Role of Complement Lectin Pathway in Trypanosomatids: Molecular Bases in Activation, Genetic Deficiencies, Susceptibility to Infection, and Complement System-Based Therapeutics

The innate immune system is evolutionary and ancient and is the pivotal line of the host defense system to protect against invading pathogens and abnormal self-derived components. Cellular and molecular components are involved in recognition and effector mechanisms for a successful innate immune response. The complement lectin pathway (CLP) was discovered in 1990. These new components at the co...