C1288
Complement C6 deficient serum human
for complement assays
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About This Item
UNSPSC Code:
12352202
NACRES:
NA.61
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Application
Complement C6 is one of the end terminals of the complement system contained in the membrane attack complex (MAC). A deficiency of C6 may result in an increased susceptibility to Neisseria meningitidis. Research has identified that a mutation which leads to a 31 bp deletion in exon 10 in the C6 gene results in C6 deficiency. A coagulation defect has also been observed in mice that are C6 deficient.
Physical form
Supplied as a solution in PBS, pH 7.4
Analysis Note
C6 is depleted by immunoadsorption as judged by a highly sensitive hemolytic assay.
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.
Storage Class
10 - Combustible liquids
wgk_germany
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
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M R Haeney et al.
Clinical and experimental immunology, 40(1), 16-24 (1980-04-01)
Isolated genetic deficiencies of complement components in man are rare. We describe two kindreds with inborn deficiencies of either C5 or C6 in which both propositi presented with recurrent bacterial meningitis. Neisseria meningitidis was isolated from the cerebrospinal fluid of
Molecular basis for complement component 6 (C6) deficiency in rats and mice.
Deepak Bhole., et al.
Immunobiol., 209, 559-568 (2004)
Kelly L Parham et al.
Molecular immunology, 44(10), 2756-2760 (2007-01-30)
Complement component C6 is one of five terminal complement components incorporated into the membrane attack complex. Complete deficiency of C6 (C6Q0) leads to an increased susceptibility to Neisseria meningitidis infections, and affected individuals typically present with recurrent meningococcal disease. There
Cryo-EM structures of Trypanosoma brucei gambiense ISG65 with human complement C3 and C3b and their roles in alternative pathway restriction.
S??lzen, et al.
Nature Communications, 14, 2403-2403 (2023)
Mahya Dezfouli et al.
Frontiers in immunology, 11, 455-455 (2020-04-08)
The clinical outcomes of primary immunodeficiencies (PIDs) are greatly improved by accurate diagnosis early in life. However, it is not common to consider PIDs before the manifestation of severe clinical symptoms. Including PIDs in the nation-wide newborn screening programs will
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