CST7 is a secreted protein and primarily expressed in peripheral blood cells and spleen.It is belongs to the cystatin family. The cystatin superfamily encompasses proteins that contain multiple cystatin-like sequences. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired this inhibitory activity. There are three inhibitory families in the superfamily, including the type 1 cystatins (stefins), type 2 cystatins and the kininogens. The type 2 cystatin proteins are a class of cysteine proteinase inhibitors found in a variety of human fluids and secretions. This gene encodes a glycosylated cysteine protease inhibitor with a putative role in immune regulation through inhibition of a unique target in the hematopoietic system.
Human Cystatin A (CSTA) is a member of family 1 of the cystatin superfamily; which is characterized by lacking of disulphide bonds an carbohydrates. Cystatin A is an intracellular inhibitor regulating the activities of cysteine proteases of the papain family such as Cathepsins B; H and L. Cystatin A is also implicated in a number of disease states. Due to altered proteolytic state in cancer progression; Cystatin A may play a role in the proteolytic pathways.
Cystatin-B, also known as CPI-B, Liver thiol proteinase inhibitor, Stefin-B, CSTB and CST6, is a cytoplasm and nucleus protein which belongs to thecystatin family. Cystatin-B / CSTB is an intracellular thiol proteinase inhibitor. Tightly binding reversible inhibitor of cathepsins L, H and B. Cystatin-B / CSTB is able to form a dimer stabilized by noncovalent forces, inhibiting papain and cathepsins l, h and b. Cystatin-B / CSTB is also thought to play a role in protecting against the proteases leaking from lysosomes. Defects in Cystatin-B / CSTB are the cause of progressive myoclonic epilepsy type 1 (EPM1) which is an autosomal recessive disorder characterized by severe, stimulus-sensitive myoclonus and tonic-clonic seizures. The cystatins are a family of cysteine protease inhibitors with homology to chicken cystatin. Cystatins are physiological inhibitors of cysteine proteinases which are widely distributed in human tissues and fluids. Cystatins typically comprise about 115 amino acids, are largely acidic, contain four conserved cysteine residues known to form two disulfide bonds. Cystatins may be glycosylated and / or phosphorylated, with similarity to fetuins, kininogens, stefins, histidine-rich glycoproteins and cystatin-related proteins. Some of the members are active cysteine protease inhibitors, while others have lost or perhaps never acquired inhibitory activity. Cystatins mainly inhibit peptidases belonging to peptidase families C1 (papain family) and C13 (legumain family).
Cystatin C is a member of family 2 of the cystatin superfamily. It is ubiquitous in human tissues and body fluids and mainly used as a biomarker of kidney function. Cystatin C inhibits many cysteine proteases such as papain and Cathepsins B; H; K; L and S. As an inhibitor of cysteine proteinases; Cystatin C is thought to serve an important physiological role as a local regulator of this enzyme activity. Recently; it has been studied for its role in predicting new-onset or deteriorating cardiovascular disease. It also seems to play a role in brain disorders involving amyloid (a specific type of protein deposition); such as Alzheimer's disease.
Cystatin C is a member of family 2 of the cystatin superfamily. It is ubiquitous in human tissues and body fluids and mainly used as a biomarker of kidney function. Cystatin C inhibits many cysteine proteases such as papain and Cathepsins B, H, K, L and S. As an inhibitor of cysteine proteinases, Cystatin C is thought to serve an important physiological role as a local regulator of this enzyme activity. Recently, it has been studied for its role in predicting new-onset or deteriorating cardiovascular disease. It also seems to play a role in brain disorders involving amyloid (a specific type of protein deposition), such as Alzheimer's disease.
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