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Merck
CN
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主要文件

安全信息

C1038

Sigma-Aldrich

Complement C4 deficient serum from guinea pig

for complement assays

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About This Item

UNSPSC代码:
12352202
NACRES:
NA.61

生物来源

guinea pig

质量水平

表单

solution

技术

activity assay: suitable

运输

dry ice

储存温度

−70°C

应用

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.

分析说明

C4 is deficient as judged by a highly sensitive hemolytic assay and Ouchterlony immunodiffusion method.

储存分类代码

10 - Combustible liquids

WGK

WGK 3

闪点(°F)

Not applicable

闪点(°C)

Not applicable

个人防护装备

Eyeshields, Gloves

法规信息

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分析证书(COA)

Lot/Batch Number

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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
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)
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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