Complement factor I deficiency
نویسندگان
چکیده
منابع مشابه
A family with complement factor I deficiency.
A family with inherited factor I deficiency is described. The proband was a 19-year-old Caucasian female with one episode of meningococcal meningitis and one episode of suspected septicaemia of unknown cause. Two obligate and two probable heterozygotes with factor I levels below the lower limit of the reference range were identified. None of these exhibited increased susceptibility to infectiou...
متن کاملA family with complement factor D deficiency
The complement system consists of more than 20 plasma proteins and plays an important role in the defense against microorganisms (1). Activation of the classical or the alternative pathway results in the cleavage of C3, the central component of the system. In the alternative pathway, the spontaneous hydrolysis product iC3 (i.e., C3 with intact peptide chains but with a hydrolyzed thioester) com...
متن کاملComplement factor 2 deficiency: serological family study
Inherited complement deficiencies are associated with a variety of connective tissue diseases. A family with inherited deficiency of complement factor 2 (C2) is described in which two family members with homozygous C2 deficiency developed cutaneous vasculitis and siccasyndrome. The otherfamily members had heterozygous C2 deficiency and each member had the HLA-A25, B18, DR2 (w15) haplotype. The ...
متن کاملC3 and C4 Complement Levels in Iron Deficiency Anemia
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 ...
متن کاملComplement factor H deficiency accelerates development of lupus nephritis.
Complement factor H (CfH) is a key regulator of the alternative pathway, and its presence on mouse platelets and podocytes allows the processing of immune complexes. Because of the role of immune complexes in the pathophysiology of lupus nephritis, we studied the role of CfH in the development of nephritis in MRL-lpr mice, an animal model of lupus. At 12 weeks, CfH-deficient MRL-lpr mice had si...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Neurology - Neuroimmunology Neuroinflammation
سال: 2020
ISSN: 2332-7812
DOI: 10.1212/nxi.0000000000000689