Cystatin C

Cystatin C, encoded by CST3, is the best-known type 2 cystatin: a small secreted inhibitor produced at a constant rate by nearly all nucleated cells and present in every body fluid. It potently inhibits cathepsins B, K, L, S, and H, helping to restrain extracellular proteolysis in tissues and inflammatory sites. Because its serum concentration depends almost entirely on glomerular filtration, cystatin C is an established clinical biomarker of kidney function. A point mutation in CST3 causes hereditary cerebral hemorrhage with amyloidosis of the Icelandic type. Recombinant cystatin C supports GFR-assay development and protease-inhibition studies.