Purified anti-Cystatin C Antibody anti-Cystatin C - E4C6(2A10H7),BioLegend,667204

Cystatin C was initially identified in human urine and was named gamma-trace. It is a type II cystatin sub-family member. The cystatin family contains three subfamilies: Type 1 cystatins, also known as stefins, which are predominantly present in the cytosol and the nuclei; Type 2 cystatins are mainly secreted proteins; Type 3 cystatins are multi-domain proteins of high molecular weight (60-120 kD) and are also known as kininogens. Cystatin C inhibits cysteine proteases from the papain family and legumain. It forms tight non-covalent associations with its target enzymes. Cystatin C antagonizes TGF-β signaling in normal and cancer cellsin vitro. In this process, Cystatin C binds to the TGF-β type II receptor and inhibits the binding of TGF-β. Cystatin C influences the progression of diseases such as, cancer metastasis, atherosclerosis, aortic aneurisms, emphysema, and arthritis. Also, it is associated with neurodegenerative disease and cerebral angiopathy. Human Cystatin C has the tendency to dimerize in order to produce very tight two-fold symmetric dimers. This amyloidogenic property suggests a mechanism for its aggregation in the brain arteries of elderly people with amyloid angiopathy. The L68Q mutant of human Cystatin C is responsible for conformational disease, which causes massive amyloidosis, cerebral hemorrhage, and death in young adults.