Description
Key facts
Cell type:A549,
Species or organism:Human,
Tissue:Lung,
Form:LiquidSee storage information,
Knockout validation:Sanger Sequencing,
Mutation description:Knockout achieved by using CRISPR/Cas9, 1 bp insertion in exon 2 and 2 bp deletion in exon 2,
Disease:Carcinoma
Product details:
We will provide viable cells that proliferate on revival.
This product is subject to limited use licenses from The Broad Institute, ERS Genomics Limited and Sigma-Aldrich Co. LLC, and is developed with patented technology. For full details of the licenses and patents please refer to our
limited use license
patent pages
Properties and Storage Information:
Gene name-CFHR3, Gene editing type-Knockout, Gene editing method-CRISPR technology, Knockout validation-Sanger Sequencing, Shipped at conditions-Dry Ice, Appropriate short-term storage conditions--196°C, Appropriate long-term storage conditions--196°C
Supplementary Information:
This supplementary information is collated from multiple sources and compiled automatically.
Complement factor H-related protein 3 (CFHR3) also known as FHR-3 plays a role in modulating the complement system. This protein has a mass of approximately 42 kDa. CFHR3 is expressed in tissues like the liver and can be found circulating in the plasma. It belongs to the factor H protein family which participates in maintaining immune homeostasis by regulating complement activation.
Biological function summary
CFHR3 acts as an antagonist to complement factor H by competing for binding sites on target surfaces and complement components. It does not function as a standalone protein but interacts closely within a family that includes CFH and other complement factor H-related proteins. CFHR3 can potentially form complexes with similar proteins to help finely tune the activity of the complement system to prevent excessive immune response.
Pathways
CFHR3 is a part of the complement cascade system specifically the alternative pathway. It contributes to the negative regulation of this pathway which is important for preventing overactivation that could harm host tissues. The protein influences the binding of complement proteins like C3b an essential component in the complement cascade and interacts with other factor H-related proteins such as CFHR1 to modulate pathway activities.
CFHR3's regulatory role connects it to conditions like atypical hemolytic uremic syndrome (aHUS) and age-related macular degeneration (AMD). In aHUS mutations or deletions in CFHR3 sometimes in conjunction with CFHR1 can lead to uncontrolled complement activation contributing to disease progression. Similarly variations in CFHR3's sequence can affect AMD risk often in association with other complement regulatory proteins including CFH indicating its significant position in disease susceptibility through its regulatory function within the immune system.
