C8195
Complement C4 from human serum
≥300,000 C4H50 units/mg protein
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About This Item
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biological source
human serum
Quality Level
form
solution
specific activity
≥300,000 C4H50 units/mg protein
technique(s)
activity assay: suitable
UniProt accession no.
shipped in
dry ice
storage temp.
−70°C
Gene Information
human ... C4A(720)
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General description
Complement C4 is mapped to human chromosome 6p21.33. It exists in two isoforms as c4A and c4B. C4 is a 200 kDa protein belonging to α2 macroglobulin (α2M) family. It undergoes posttranslational modification like sulfation and glycosylation and finally proteolysis to become a mature protein.
Application
Complement C4 from human serum has been used in mannose binding lectin (MBL) complex assay of serum samples and in MBL-associated serine proteases based C4 activation assay of plasma samples.
Biochem/physiol Actions
Complement C4 serves a primary role in the classical and lectin pathways of the complement system and provides protection against bacterial pathogens. C4 polymorphisms have been used in research to study their associations with the hemolytic and antibacterial ability of the classical pathway.
Variation in the copy number of the complement C4 gene alters the complement c4A and c4B levels in age-related macular degeneration (AMD) and increases risk of developing schizophrenia.
There are strong indications that complement C4 deficiency is correlated with induction of systemic lupus erythematosus (SLE) and other autoimmune diseases.
Physical form
Supplied as a solution in PBS, pH 7.2
Other Notes
View more information on the complement pathway at www.sigma-aldrich.com/enzymeexplorer
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 Code
10 - Combustible liquids
WGK
WGK 2
Flash Point(F)
Not applicable
Flash Point(C)
Not applicable
Certificates of Analysis (COA)
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Yan Yang et al.
Current directions in autoimmunity, 7, 98-132 (2004-01-15)
It was observed about 50 years ago that low serum complement activity or low protein concentrations of complement C4 concurred with disease activities of systemic lupus erythematosus (SLE). Complete deficiencies of complement components C4A and C4B, albeit rare in human
Genetic, structural and functional diversities of human complement components C4A and C4B and their mouse homologues, Slp and C4
Blanchong CA, et al.
International Immunopharmacology, 1(3), 365-392 (2001)
Low C4 gene copy numbers are associated with superior graft survival in patients transplanted with a deceased donor kidney
Bay JT, et al.
Kidney International, 84(3), 562-569 (2013)
Schizophrenia risk from complex variation of complement component 4
Sekar A, et al.
Nature, 530(7589), 177-177 (2016)
Anti-MBL autoantibodies in patients with rheumatoid arthritis: prevalence and clinical significance
Gupta B, et al.
Journal of Autoimmunity, 27(2), 125-133 (2006)
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