C1163
Complement C5 deficient serum human
for complement assays
Synonym(s):
Complement serum
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About This Item
NACRES:
NA.61
UNSPSC Code:
12352202
Analysis Note
C5 is depleted by immunoadsorption as determined by hemolytic assay.
Application
Complement C5 deficiencies in humans are rare, but often associated with recurrent infections by Neisseria spp. (such as meningitis). Deficiency in C5 results in an impairment of hemolytic activity from both the classical and alternative pathway of the complement system. Recently, genetic research has revealed that a 153 bp deletion in exon 30 results in complete C5 deficiency. Research has also implicated C5 deficiency in a predisposition to cardiac dysfunction when cardiac injury occurs.
Biochem/physiol Actions
Complement C5 deficiency can, paradoxically, protect against certain effects of infection. Patients deficient in C5 are unusually susceptible to meningococcal infection, but have a milder course of infection. Reduced expression of tumor necrosis factor (TNF) may account for this effect.
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 7.4
Storage Class
12 - Non Combustible Liquids
wgk
WGK 3
flash_point_f
Not applicable
flash_point_c
Not applicable
Regulatory Information
高风险级别生物产品--人源产品
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Taku Fukuzawa et al.
Scientific reports, 7(1), 1080-1080 (2017-04-26)
Dysregulation of the complement system is linked to the pathogenesis of a variety of hematological disorders. Eculizumab, an anti-complement C5 monoclonal antibody, is the current standard of care for paroxysmal nocturnal hemoglobinuria (PNH) and atypical hemolytic uremic syndrome (aHUS). However
A novel role for the fifth component of complement (C5) in cardiac physiology.
Mullick A., et al.
PLoS ONE, 6 (2011)
Kelly Mulfaul et al.
The Journal of pathology, 257(1), 29-38 (2022-01-18)
Activation of the alternative complement pathway is an initiating event in the pathology of age-related macular degeneration (AMD). Unchecked complement activation leads to the formation of a pro-lytic pore, the membrane attack complex (MAC). MAC deposition is observed on the
P A Barton et al.
Infection and immunity, 61(4), 1474-1481 (1993-04-01)
Patients with disseminated Neisseria meningitidis infections (meningococcemia) suffer from a fulminant shock syndrome that is accompanied by extraordinarily high concentrations in serum of tumor necrosis factor (TNF). People with homozygous deficiencies of late complement components (C5, C6, C7, and C8)
P Aguilar-Ramirez et al.
Molecular immunology, 46(10), 2116-2123 (2009-04-21)
The deficiency of complement C5 is rare and frequently associated with severe and recurrent infections, especially caused by Neisseria spp. We observed the absence of component C5 in the serum of 3 siblings from a Brazilian family with history of
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