Cystatin C Protein | CST3 protein
Cystatin C (Cystatin 3) Antigen, Human Urine
Cystatin C is a marker for allograph function in adult transplant patients. Cystatin C regulates extracellular cysteine protease activity, which results from microbial invasion or release of lysosomal proteinases from dying or diseased cells. It is freely filtered by the glomerular basement membrane (GBM) and serum concentration has been shown to correlate well with glomerular filtration rate (GFR).
NCBI and Uniprot Product Information
NCBI Description
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, where they appear to provide protective functions. The cystatin locus on chromosome 20 contains the majority of the type 2 cystatin genes and pseudogenes. This gene is located in the cystatin locus and encodes the most abundant extracellular inhibitor of cysteine proteases, which is found in high concentrations in biological fluids and is expressed in virtually all organs of the body. A mutation in this gene has been associated with amyloid angiopathy. Expression of this protein in vascular wall smooth muscle cells is severely reduced in both atherosclerotic and aneurysmal aortic lesions, establishing its role in vascular disease. In addition, this protein has been shown to have an antimicrobial function, inhibiting the replication of herpes simplex virus. Alternative splicing results in multiple transcript variants encoding a single protein. [provided by RefSeq, Nov 2014]
Uniprot Description
CST3: As an inhibitor of cysteine proteinases, this protein is thought to serve an important physiological role as a local regulator of this enzyme activity. Defects in CST3 are the cause of amyloidosis type 6 (AMYL6); also known as hereditary cerebral hemorrhage with amyloidosis (HCHWA), cerebral amyloid angiopathy (CAA) or cerebroarterial amyloidosis Icelandic type. AMYL6 is a hereditary generalized amyloidosis due to cystatin C amyloid deposition. Cystatin C amyloid accumulates in the walls of arteries, arterioles, and sometimes capillaries and veins of the brain, and in various organs including lymphoid tissue, spleen, salivary glands, and seminal vesicles. Amyloid deposition in the cerebral vessels results in cerebral amyloid angiopathy, cerebral hemorrhage and premature stroke. Cystatin C levels in the cerebrospinal fluid are abnormally low. Genetic variations in CST3 are associated with age- related macular degeneration type 11 (ARMD11). ARMD is a multifactorial eye disease and the most common cause of irreversible vision loss in the developed world. In most patients, the disease is manifest as ophthalmoscopically visible yellowish accumulations of protein and lipid that lie beneath the retinal pigment epithelium and within an elastin-containing structure known as Bruch membrane. Belongs to the cystatin family.
Protein type: Secreted; Secreted, signal peptide; Inhibitor
Chromosomal Location of Human Ortholog: 20p11.21
Cellular Component: extracellular space; extracellular region
Molecular Function: protein binding; protease binding; beta-amyloid binding; endopeptidase inhibitor activity; cysteine protease inhibitor activity
Biological Process: negative regulation of proteolysis; fibril organization and biogenesis; negative regulation of peptidase activity; defense response; regulation of tissue remodeling
Disease: Macular Degeneration, Age-related, 11; Cerebral Amyloid Angiopathy, Cst3-related
Research Articles on CST3
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Product Notes
The CST3 cst3 (Catalog #AAA173070) is a Protein produced from Human urine and is intended for research purposes only. The product is available for immediate purchase. It is sometimes possible for the material contained within the vial of "Cystatin C, Protein" to become dispersed throughout the inside of the vial, particularly around the seal of said vial, during shipment and storage. We always suggest centrifuging these vials to consolidate all of the liquid away from the lid and to the bottom of the vial prior to opening. Please be advised that certain products may require dry ice for shipping and that, if this is the case, an additional dry ice fee may also be required.Precautions
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