C57BL/6JCya-Cd46em1flox/Cya
Common Name:
Cd46-flox
Product ID:
S-CKO-03685
Background:
C57BL/6JCya
Product Type
Age
Genotype
Sex
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Basic Information
Strain Name
Cd46-flox
Strain ID
CKOCMP-17221-Cd46-B6J-VA
Gene Name
Product ID
S-CKO-03685
Gene Alias
Mcp
Background
C57BL/6JCya
NCBI ID
Modification
Conditional knockout
Chromosome
1
Phenotype
Document
Application
--
Note: When using this mouse strain in a publication, please cite “C57BL/6JCya-Cd46em1flox/Cya mice (Catalog S-CKO-03685) were purchased from Cyagen.”
Strain Description
Ensembl Number
ENSMUST00000162650
NCBI RefSeq
NM_010778.4
Target Region
Exon 2~3
Size of Effective Region
~3.0 kb
Detailed Document
Overview of Gene Research
Cd46, also known as membrane cofactor protein (MCP), is a ubiquitously expressed complement regulatory protein. It serves as a cofactor for serine protease factor I to cleave and inactivate C3b and C4b deposited on host cells, playing a crucial role in preventing complement-mediated "self-attack" [1,3]. Additionally, it is involved in human reproduction, autophagy, modulating T cell activation and effector functions, and is part of the intracellular complement system (complosome) [1]. It is also a receptor for 11 pathogens, earning it the moniker "pathogen magnet" [1].
Mutations in Cd46 predispose to atypical hemolytic uremic syndrome (aHUS) with low penetrance. In a study of a large single family, individuals carrying a rare, heterozygous Cd46 gene splice-site mutation had reduced Cd4 + T cell-intrinsic Cd46 expression. The diseased mutation carriers had lower peripheral regulatory T cell (Treg) frequencies and carried potentially epistatic, private rare variants in other inborn errors of immunity (IEI)-associated proinflammatory genes compared to healthy carriers, suggesting that these factors may contribute to clinically manifest Cd46 haploinsufficiency-associated immune-dysregulation [2].
In conclusion, Cd46 is essential in complement regulation, immune modulation, and pathogen interaction. Its study through genetic models, as in the case of the family-based analysis of Cd46 mutations, helps in understanding its role in diseases like aHUS and immune-dysregulation. This knowledge provides insights into the mechanisms underlying these disease conditions and may guide the development of new therapeutic strategies.
References:
1. Liszewski, M Kathryn, Atkinson, John P. 2021. Membrane cofactor protein (MCP; CD46): deficiency states and pathogen connections. In Current opinion in immunology, 72, 126-134. doi:10.1016/j.coi.2021.04.005. https://pubmed.ncbi.nlm.nih.gov/34004375/
2. Meyer, Benedikt J, Kunz, Natalia, Seki, Sayuri, Hess, Christoph, Recher, Mike. 2023. Immunologic and Genetic Contributors to CD46-Dependent Immune Dysregulation. In Journal of clinical immunology, 43, 1840-1856. doi:10.1007/s10875-023-01547-y. https://pubmed.ncbi.nlm.nih.gov/37477760/
3. Cardone, J, Le Friec, G, Kemper, C. 2011. CD46 in innate and adaptive immunity: an update. In Clinical and experimental immunology, 164, 301-11. doi:10.1111/j.1365-2249.2011.04400.x. https://pubmed.ncbi.nlm.nih.gov/21488871/
Quality Control Standard
Sperm Test
Pre-cryopreservation: Measurement of sperm concentration, determination of sperm viability.
Post-cryopreservation: A vial of cryopreserved sperms is selected for in-vitro fertilization from each batch.
Environmental Standards:SPF
Available Region:Global
Source:Cyagen