Ads
related to: c1 inhibitor protein
Search results
Results From The WOW.Com Content Network
C1-inhibitor is the largest member among the serpin superfamily of proteins. It can be noted that, unlike most family members, C1-inhibitor has a 2-domain structure.The C-terminal serpin domain is similar to other serpins, which is the part of C1-inhibitor that provides the inhibitory activity.
The role of C1-INH is to regulate and control the activities of the complement cascade, such that complement proteins remain in check and do not lead to unnecessary activity. [4] When there is a deficiency of C1-INH due to one of the previously mentioned causes, then the complement cascade remains continuously activated and can lead to ...
The C1 complex (complement component 1, C1) is a protein complex involved in the complement system. It is the first component of the classical complement pathway and is composed of the subcomponents C1q, C1r and C1s. [1] [2] [3]
In HAE type II, there is a qualitative deficiency, with normal - sometimes even elevated - C1-inhibitor protein levels, but decreased functional C1-inhibitor measurements. This type is seen in approximately 15% of HAE cases with C1-inhibitor deficiency. [16] [17]
Disorders of the proteins that act to inhibit the complement system (such as C1-inhibitor) can lead to an overactive response, causing conditions such as hereditary angioedema. [8] Disorders of the proteins that act to activate the complement system (such as C3) can lead to an underactive response, causing greater susceptibility to infections. [9]
C1-inhibitor plays the role of inactivating C1r and C1s to prevent further downstream classical complement activity. [13] [12] C1-inhibitor controls the processes involved in maintaining vascular permeability. As a result, C1-inhibitor levels of less than 50% of the standard lead to increased vascular permeability, characteristic of angioedema ...