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Merck
CN

C1538

Complement C8 deficient serum 人

for complement assays

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关于此项目

NACRES:
NA.61
UNSPSC Code:
12352202
Biological source:
human
Origin:
USA origin
Form:
solution
Shipped in:
dry ice
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产品名称

Complement C8 deficient serum 人, for complement assays

biological source

human

form

solution

origin

USA origin

technique(s)

activity assay: suitable

impurities

infectious agent, tested

UniProt accession no.

shipped in

dry ice

storage temp.

−70°C

Quality Level

Gene Information

human ... C8A(731)

Analysis Note

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

Application

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.

Biochem/physiol Actions

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.

Disclaimer

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.

Physical form

Supplied as a solution in PBS, pH 7.3

存储类别

10 - Combustible liquids

wgk

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable

法规信息

新产品
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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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