Rabbit CST3 Polyclonal Antibody | anti-CST3 antibody
CST3 Polyclonal Antibody
IHC: 1:100-1:300
Western Blot (WB)
(Western Blot analysis of Caco-2 cells using CST3 Polyclonal Antibody at dilution of 1:3000)
Immunohistochemistry (IHC)
(Immunohistochemistry of paraffin-embedded Human kidney using CST3 Polycloanl Antibody at dilution of 1:200)
Immunohistochemsitry (IHC)]
(Immunohistochemistry of paraffin-embedded Mouse kidney using CST3 Polycloanl Antibody at dilution of 1:200)
Immunohistochemistry (IHC)
(Immunohistochemistry of paraffin-embedded Rat kidney using CST3 Polycloanl Antibody at dilution of 1:200)
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, signal peptide; Inhibitor; Secreted
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
