B6-hC3 Mouse
Product Name
B6-hC3 Mouse
Product ID
I001135
Strain Name
C57BL/6JCya-C3tm1(hC3)/Cya
Backgroud
C57BL/6JCya
Note
“B6-hC3,” “B6-huC3,” “hC3,” and “huC3” all denote the same strain throughout the text and data figures.
When using this mouse strain in a publication, please cite “B6-hC3 Mouse (Catalog I001135) were purchased from Cyagen.”
Product Type
Age
Genotype
Sex
Quantity
Gene Name
Gene Alias
ASP, C3a, C3b, AHUS5, ARMD9, CPAMD1, HEL-S-62p
NCBI ID
Chromosome
Chr 19
MGI ID
Datasheet
Strain Description
Complement component C3 plays a central role in activating the complement system and is the most abundant complement protein in human plasma, primarily synthesized in the liver. As part of the innate immune system, the complement system is activated during tissue damage and pathogen invasion, playing a crucial role in the inflammatory response, host homeostasis, and pathogen defense. The complement cascade is activated through the classical pathway, alternative pathway, and lectin pathway, all of which generate C3 convertase, which cleaves C3 into C3a and C3b. C3a is a potent anaphylatoxin with pro-inflammatory activity, while C3b is a regulator that induces C5 cleavage, thereby participating in the dissolution and clearance of immune complexes. Mutations in this gene are associated with atypical hemolytic uremic syndrome (aHUS) and age-related macular degeneration (AMD). Deficiencies in C3 and C3-derived peptides can lead to autoimmune diseases (such as rheumatoid arthritis, systemic lupus erythematosus, and vasculitis) and make individuals susceptible to recurrent respiratory infections and infections caused by encapsulated organisms. Conversely, excessive activation of C3 and related complement components is associated with kidney diseases (immune complex glomerulonephritis, hemolytic uremic syndrome, lupus nephritis, membranous nephropathy, and immune-mediated nephropathy) [1-2].
The B6-huC3 mouse is a mouse C3 humanized model created by replacing the mouse C3 gene with the human C3 gene using gene-editing technology. The humanized regions include the 5’UTR and 3’UTR. Under natural breeding conditions, homozygous B6-huC3 mice exhibit mortality around 10 weeks of age, and it has been observed that heterozygous mice also experience mortality. Additionally, based on the innovative TurboKnockout technology combined with BAC recombination developed by Cyagen Biosciences, customized services are available for different mutations to meet the experimental needs of researchers studying complement-mediated diseases.
Reference
Delanghe JR, Speeckaert R, Speeckaert MM. Complement C3 and its polymorphism: biological and clinical consequences. Pathology. 2014 Jan;46(1):1-10.
Yates JR, Sepp T, Matharu BK, Khan JC, Thurlby DA, Shahid H, Clayton DG, Hayward C, Morgan J, Wright AF, Armbrecht AM, Dhillon B, Deary IJ, Redmond E, Bird AC, Moore AT; Genetic Factors in AMD Study Group. Complement C3 variant and the risk of age-related macular degeneration. N Engl J Med. 2007 Aug 9;357(6):553-61.
Strain Strategy
Figure 1. Gene editing strategy of B6-huC3 mice. The sequences from upstream of exon 1 to the TGA stop codon of mouse C3 were replaced with the sequences from upstream of exon 1 to downstream of exon 41 of human C3.
Application Area
Preclinical research on C3-targeted drugs;
Research in immunotherapy, oncology, etc.
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