Cystatin C (rCST3) Rat Recombinant Protein

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, signal peptide; Inhibitor; Secreted

Cellular Component: extracellular space; nuclear membrane; contractile fiber; lysosome; endoplasmic reticulum; extracellular region; multivesicular body; cell projection; cell soma; perinuclear region of cytoplasm; axon; cytoplasm; basement membrane; vesicle

Molecular Function: protease binding; protease inhibitor activity; beta-amyloid binding; endopeptidase inhibitor activity; cysteine protease inhibitor activity

Biological Process: salivary gland development; apoptosis; response to toxin; negative regulation of peptidase activity; defense response; response to organic cyclic substance; response to carbohydrate stimulus; response to estradiol stimulus; positive regulation of cell proliferation; fibril organization and biogenesis; circadian sleep/wake cycle, REM sleep; response to axon injury; negative regulation of proteolysis; response to drug; response to nutrient levels; response to inorganic substance; male gonad development; regulation of programmed cell death; Sertoli cell development; cell activation; eye development; response to hypoxia; brain development; response to oxidative stress; positive regulation of DNA replication; embryo implantation; regulation of tissue remodeling