primary complement deficiencies are rare and two related patients are reported here. the first patient is a 41- year- old man with eighteen episodes of pneumo¬coccal meningitis and other purulent infections. the serum c3 level was checked at three separate times, showing that this was a primary c3 deficient case; other immunological tests were however normal. this patient now takes prophylactic...

background and objectives: complement proteins are some of the most important plasma proteins of the innate immune system. impaired immune function is reported in subjects who are iron deficient, and there are documents that these patients are prone to infection. this study was conducted to show whether serum c3 and c4 complement change in adult nonpregnant female with iron deficient anemia or ...

In experiments to ascertain the biochemical basis of a genetically determined deficiency of the third component of complement (C3) in guinea pigs, we found that C3-deficient liver and peritoneal macrophages contain C3 messenger RNA of normal size (approximately 5 kb) and amounts, that this mRNA programs synthesis of pro-C3 in oocytes primed with liver RNA and in primary macrophage cultures. In ...

background: primary antibody deficiency, the most common primary immunodeficiency disorder, represents a heterogeneous spectrum of conditions caused by a defect in any critical stage of b cell development and is characterized by impaired production of normal amounts of antigen-specific antibodies. objective: this retrospective study aimed at description and analysis of demographic, clinical, im...

Abstract Intracellular complement proteins such as C3 and FB play protective role during stress-induced cell death. C3−/−and Cfb−/−mice had worse Pseudomonas aeruginosa (P.a.)-induced acute lung injury at 24 h post infection compared to wildtype (WT) mice. However, the mechanism by which exerts these functions is unclear. We performed CRISPR-induced deletion of C3and CFBin BEAS-2B cells, a huma...

Primary selective immunoglobulin (Ig) M deficiency usually presents early in life with recurrent or severe infections caused by encapsulated and gram-negative organisms. Primary selective IgM deficiency in adults is rare and is usually associated with autoimmune diseases or malignant neoplasm. We performed an extensive immunological analysis of innate and adaptive immunity in an adult patient w...

The inflammatory kidney disease membranoproliferative glomerulonephritis type II (MPGN2) is associated with dysregulation of the alternative pathway of complement activation. MPGN2 is characterized by the presence of complement C3 along the glomerular basement membrane (GBM). Spontaneous activation of C3 through the alternative pathway is regulated by 2 plasma proteins, factor H and factor I. D...

Vitamin B12 deficiency seems to be more common worldwide than previously thought. However, only a few reports based on data from newborn screening (NBS) programs have drawn attention to that subject. In Estonia, over the past three years, we have diagnosed 14 newborns with congenital acquired vitamin B12 deficiency. Therefore, the incidence of that condition is 33.8/100,000 live births, which i...

Uncontrolled activation of the complement alternative pathway is associated with complement-mediated renal disease. Factor B and factor D are essential components of this pathway, while factor H (FH) is its major regulator. In complete FH deficiency, uncontrolled C3 activation through the alternative pathway results in plasma C3 depletion and complement-mediated renal disease. These are depende...

Impaired T-cell reactivity is a feature of C3-deficient mice in several disease models. The mechanism behind the reduced T-cell response is, however, poorly understood. We explored the hypothesis that antigen-presenting cells (APCs) from C3-/- mice have impaired potency to stimulate antigen-specific T cells, in an alloantigen-dependent model. Our results show that C3-/- macrophages have reduced...