C1rb (complement component 1, r subcomponent B), also known as Gm8551 or mC1rB. is a vital serine protease. It has been reported that in mice, the C1r gene duplicates to produce two isoforms, C1ra and C1rb, with vastly different tissue distributions. For example, C1ra is primarily found in the small intestine, liver, blood, and midbrain, while C1rB is found in male reproductive organs, including the prostate and coagulation glands.
Its Gene ID: 667277 and UniProtKB ID: Q8CFG9.
Similar to C1ra, C1rb also contains multiple specific domains. However, there are differences between the primary sequences of C1ra and C1rb, and this difference is mainly found in the SP domain. In addition, the scientists found that the aa sequence identity between the mouse full-length C1ra and C1rB is 96%. Mouse C1rB has 79.9% identity with human C1r. Notably, the modular structure of C1rb is the same as that of the human protein. Notably, there are differences in the distribution of amino acid residues between humans and mice, and this is prevalent in the SP domain.
C1rb combines with C1q and C1s to form C1, which serves as the first component of the classical pathway of the complement system. Specifically, activation of C1 promotes the conversion of C1r proenzyme to C1r enzyme. This signal helps activate two C1s proenzymes. C1s then combine with C4 to produce C4a and C4b, which in turn facilitates the splitting of C2 into C2a and C2b. Subsequently, C3 convertase cleaves C3 into C3a and C3b, and C3b can combine with the C4b2a combination to produce C5 convertase. Subsequently, C5 is catalyzed by C5 convertase to form C5a and C5b. C5b is associated with other complementary components to create a complex that mediates the lysis of invasive bacteria.
Fig.1 C1r and classical complement pathways.1,3
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Mouse Complement C1RB ELISA Kit
Recombinant Mouse Complement Component C1rb Protein
Mouse Complement C1rb (NM_001113356) Myc-DDK tagged ORF clone-CTDNA-023
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Creative Biolabs provides a comprehensive range of C1rb related products, including anti-C1rb ELISA kits and C1rb proteins. These products can effectively carry out C1rb related experiments and thus play an important role in your research.
Fig. 2 Effects of Sal B on mRNAs and circRNAs expression in obese mice, C1rb was the most downregulated.2,3
Marfan syndrome (MFS) is a connective tissue disorder that can lead to the formation of aortic aneurysms. Current treatments focus on preventive aortic surgery and antihypertensive medications to reduce the risk of aortic rupture. Recent whole genome sequencing of families affected by Marfan syndrome identified a variant in the complement gene C1R, which is associated with severe cases of aortic disease. Research showed that various complement genes and proteins are expressed in the aneurysm tissues of both humans and mouse models of MFS.2,3 This led to an investigation into complement inhibition in MFS mouse models. When MFS mice were treated with a human plasma-derived C1 esterase inhibitor, there was a reduction in complement deposition, decreased influx of macrophages into the aorta, and lower levels of circulating TNFα. However, the inhibition of complement did not affect the rate of aortic dilation in this MFS mouse model, indicating that complement inhibition may not be a viable therapeutic strategy for Marfan syndrome.
Creative Biolabs offers C1rb functional services, including detection of C1rb binding and other tailored functional services for our esteemed clients engaged in clinical and scientific research.
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