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Key Documents

C1163

Sigma-Aldrich

Complement C5 deficient serum human

for complement assays

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About This Item

UNSPSC Code:
12352202
NACRES:
NA.61

biological source

human

Quality Level

form

solution

technique(s)

activity assay: suitable

UniProt accession no.

storage temp.

−70°C

Gene Information

human ... C5(727)

Categorías relacionadas

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.

Physical form

Supplied as a solution in PBS, pH 7.4

Analysis Note

C5 is depleted by immunoadsorption as determined by 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

12 - Non Combustible Liquids

wgk_germany

WGK 3

flash_point_f

Not applicable

flash_point_c

Not applicable


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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)
A novel role for the fifth component of complement (C5) in cardiac physiology.
Mullick A., et al.
PLoS ONE, 6 (2011)
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
Robert Lodge et al.
PloS one, 7(3), e32761-e32761 (2012-03-14)
Over the past decade, the number of reported human immunodeficiency virus type-1 (HIV-1)/Leishmania co-infections has risen dramatically, particularly in regions where both diseases are endemic. Although it is known that HIV-1 infection leads to an increase in susceptibility to Leishmania
Alaka Mullick et al.
Infection and immunity, 74(8), 4439-4451 (2006-07-25)
The effect of a deficiency in the C5 component of complement on the pathophyisology of infection with the fungal pathogen Candida albicans was studied by using the A/J inbred mouse strain and the BcA17 congenic mouse strain. Acute infection caused

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