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Merck
CN

C1538

Sigma-Aldrich

Complement C8 deficient serum 人

for complement assays

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About This Item

UNSPSC代码:
12352202
NACRES:
NA.61
生物来源:
human
来源:
USA origin
表单:
solution
运输:
dry ice

生物来源

human

质量水平

表单

solution

来源

USA origin

技术

activity assay: suitable

杂质

infectious agent, tested

UniProt登记号

运输

dry ice

储存温度

−70°C

基因信息

human ... C8A(731)

应用

Complement C8 is one of the end terminals of the complement system contained in the membrane attack complex (MAC). A deficiency of C8 may result in an increased susceptibility to Neisseria meningitidis. The most common mutation resulting in a C8 deficiency is a C to T transition in exon 9 of the C8 beta gene. Recent research however, has also discovered that two separate heterogeneous mutations may result in C8 deficiency in patients displaying recurrent meningococcal meningitis. These mutations are a duplication mutation on exon 7 and a mutation on exon 3.

生化/生理作用

Serum naturally deficient in C8 may actually contain some C8, though in greatly reduced quantity. Terminal complement complex is present at trace levels in such sera.

外形

Supplied as a solution in PBS, pH 7.3

分析说明

C8 is depleted by immunoadsorption as judged by a highly sensitive hemolytic assay.

免责声明

RESEARCH USE ONLY. This product is regulated in France when intended to be used for scientific purposes, including for import and export activities (Article L 1211-1 paragraph 2 of the Public Health Code). The purchaser (i.e. enduser) is required to obtain an import authorization from the France Ministry of Research referred in the Article L1245-5-1 II. of Public Health Code. By ordering this product, you are confirming that you have obtained the proper import authorization.

储存分类代码

10 - Combustible liquids

WGK

WGK 3

闪点(°F)

Not applicable

闪点(°C)

Not applicable

法规信息

新产品

历史批次信息供参考:

分析证书(COA)

Lot/Batch Number

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T E Mollnes et al.
Scandinavian journal of immunology, 24(3), 307-312 (1986-09-01)
The terminal complement complex (TCC) was quantified in sera from patients with a genetic deficiency of C8 alpha-gamma or C8 beta. The individual sera contained only trace amounts of TCC compared with a normal serum pool. The content of TCC
L Saucedo et al.
Journal of immunology (Baltimore, Md. : 1950), 155(10), 5022-5028 (1995-11-15)
We studied the molecular bases for C8 beta deficiency in 34 unrelated families from the United States and the former Soviet Union. These families represented 69 unrelated null alleles of which 59 (86%) were found to be due to a
W P Kolb et al.
Journal of immunology (Baltimore, Md. : 1950), 122(5), 2103-2111 (1979-05-01)
C1q, a subcomponent of the first component of complement, has been isolated from human serum in fully hemolytically active form by affinity column chromatography and gel filtration with Bio-Gel A-5M. The affinity column was prepared by covalent coupling of purified

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