C1038
Complement C4 deficient serum from guinea pig
for complement assays
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About This Item
UNSPSC Code:
12352202
NACRES:
NA.61
Recommended Products
biological source
guinea pig
Quality Level
form
solution
technique(s)
activity assay: suitable
shipped in
dry ice
storage temp.
−70°C
Application
Complement C4 deficiency has long been associated with the disease systemic lupus erythematosus (SLE) and severe kidney disorders. Screening of the mutations which lead to C4 deficiency may assist in studies of these diseases and disorders. It has been demonstrated that 40-60% of patients with SLE contained either heterozygous or homozygous deficiencies in C4 compliment components via C4A and C4B genes. In particular, research has shown that complete C4A and C4B deficiencies occur through deleterious mutations at exon 13 and within a 2.6-kb genomic region spanning exons 20-29.
Analysis Note
C4 is deficient as judged by a highly sensitive hemolytic assay and Ouchterlony immunodiffusion method.
Storage Class
10 - Combustible liquids
wgk_germany
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
ppe
Eyeshields, Gloves
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Complete complement components C4A and C4B deficiencies in human kidney diseases and systemic lupus erythematosus.
Yang Y., et al.
Journal of Immunology, 15, 2803-2814 (2004)
Manfred Nairz et al.
Frontiers in cellular and infection microbiology, 7, 110-110 (2017-04-27)
Genetic and dietary forms of iron overload have distinctive clinical and pathophysiological features. HFE-associated hereditary hemochromatosis is characterized by overwhelming intestinal iron absorption, parenchymal iron deposition, and macrophage iron depletion. In contrast, excessive dietary iron intake results in iron deposition
Anamarija Markota et al.
PloS one, 14(7), e0218332-e0218332 (2019-07-06)
Clinical observations in inflammatory bowel disease patients and experimental studies in rodents suggest that iron in the intestinal lumen derived from iron-rich food or oral iron supplementation could exacerbate inflammation and that iron depletion from the diet could be protective.
Wannaporn Ittiprasert et al.
Journal of autoimmunity, 25(1), 77-84 (2005-07-07)
The complement component C4 is encoded by two genes: C4A and C4B on human chromosome 6p in the major histocompatibility complex (MHC). Most studies have linked the deficiencies in C4 with systemic lupus erythematosus (SLE) in Angio-Irish, North American, Black
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