Cystatin C, Inhibitor | CST3, inhibitor

Cystatin C, Human (AD8, Amyloid Angiopathy and Cerebral Hemorrhage, Cst3, Cystatin 3, Gamma Trace, HCCAA, Neuroendocrine Basic Polypeptide, Post gamma Globulin)

Cat. Num. AAA655365

• Gene Names: CST3; ARMD11
• Applications: ELISA, Western Blot
• Purity: ~90% SDS-PAGE
• Synonyms: Cystatin C; Human (AD8; Amyloid Angiopathy and Cerebral Hemorrhage; Cst3; Cystatin 3; Gamma Trace; HCCAA; Neuroendocrine Basic Polypeptide; Post gamma Globulin); CST3; inhibitor

• Host: Human urine
• Purity/Purification: ~90% SDS-PAGE
• Form/Format: Supplied as a lyophilized powder from ammonium bicarbonate pH 4.5. May contain traces of buffer salts. Reconstitute in 0.02M ammonium bicarbonate buffer.

• Applicable Applications for CST3, inhibitor: ELISA (EL/EIA), Western Blot (WB)
• Application Notes: Suitable for use in ELISA and Western Blot.
• Preparation and Storage: Lyophilized powder may be stored at -20 degree C. Stable for 12 months at -20 degree C. Reconstitute with sterile 0.02M ammonium bicarbonate buffer. Aliquot to avoid repeated freezing and thawing. Store at -20 degree C. For maximum recovery of product, centrifuge the original vial after thawing and prior to removing the cap. Further dilutions can be made in assay buffer.

Related Product Information for CST3, inhibitor

Cystatins are a superfamily of cysteine proteinase inhibitors found in both plants and animals. They comprise a group of proteinase inhibitors, widely distributed in tissues and body fluids, and form tight complexes with cysteine proteases such as cathepsin B, H, L and S. Cystatin C, a secreted molecule of this family, is of interest from biochemical, medicine and evolutionary points of view. Cystatin C, with molecular weight of 13.26kD, is composed of 120 amino acids, lacks carbohydrate and has two disulfide bridges located near the carboxyl terminus. Cystatin C is increased in patients with malignant diseases, and is related to the insufficiency of renal function and appears to be a better marker than creatinine. On the other hand, low levels of cystatin C involve cause the breakdown of the elastic laminae and, subsequently, the atherosclerosis and abdominal aortic aneurysm.

Product Categories/Family for CST3, inhibitor

Molecular Biology; MB-Protease Inhibitors

NCBI and Uniprot Product Information

• NCBI GI #: 755738
• NCBI GeneID: 1471
• UniProt Accession #: P01034; Q6FGW9; B2R5J9; D3DW42
• Molecular Weight: 13.26kD
• NCBI Official Full Name: cystatin C
• NCBI Official Synonym Full Names: cystatin C
• NCBI Official Symbol: CST3
• NCBI Official Synonym Symbols: ARMD11
• NCBI Protein Information: cystatin-C; cystatin 3; cystatin-3; gamma-trace; post-gamma-globulin; bA218C14.4 (cystatin C); neuroendocrine basic polypeptide
• UniProt Protein Name: Cystatin-C
• Protein Family: Cystatin
• UniProt Gene Name: CST3
• UniProt Entry Name: CYTC_HUMAN

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. [provided by RefSeq, Jul 2008]

Uniprot Description

Function: As an inhibitor of cysteine proteinases, this protein is thought to serve an important physiological role as a local regulator of this enzyme activity.

Subunit structure: Homodimer.

Subcellular location: Secreted Ref.19.

Tissue specificity: Expressed in submandibular and sublingual saliva but not in parotid saliva (at protein level). Expressed in various body fluids, such as the cerebrospinal fluid and plasma. Expressed in highest levels in the epididymis, vas deferens, brain, thymus, and ovary and the lowest in the submandibular gland. Ref.16 Ref.19

Post-translational modification: The Thr-25 variant is O-glycosylated with a core 1 or possibly core 8 glycan. The signal peptide of the O-glycosylated Thr-25 variant is cleaved between Ala-20 and Val-21.

Involvement in disease: Amyloidosis 6 (AMYL6) [MIM:105150]: 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.Note: The disease is caused by mutations affecting the gene represented in this entry. Ref.3 Ref.23Age-related macular degeneration 11 (ARMD11) [MIM:611953]: A form of age-related macular degeneration, 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.Note: Disease susceptibility is associated with variations affecting the gene represented in this entry. Ref.24

Miscellaneous: Potential cerebrospinal fluid marker for the diagnosis of Creutzfeldt-Jakob disease.

Sequence similarities: Belongs to the cystatin family.

Mass spectrometry: Molecular mass is 13334.5829±0.0140 Da from positions 27 - 146. Determined by ESI. Ref.19