Summary: 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].

cystatin CMIM:604312Ensembl:ENSG00000101439HGNC:HGNC:2475PA2697620p11.21

GO terms in CST3

Term TypeEvidence TypeGO Term IDGO Des.
MFIPIGO:0001540amyloid-beta binding
MFIPIGO:0002020protease binding
MFIDAGO:0004866endopeptidase inhibitor activity
MFIDAGO:0004869cysteine-type endopeptidase inhibitor activity
MFIPIGO:0005515protein binding
MFIPIGO:0042802identical protein binding
CCIMPGO:0005576extracellular region
CCTASGO:0005576extracellular region
CCIEAGO:0005604basement membrane
CCHDAGO:0005615extracellular space
CCIBAGO:0005615extracellular space
CCIDAGO:0005615extracellular space
CCIEAGO:0005771multivesicular body
CCTASGO:0005788endoplasmic reticulum lumen
CCIEAGO:0031965nuclear membrane
CCIEAGO:0043025neuronal cell body
CCIEAGO:0043292contractile fiber
CCIEAGO:0048471perinuclear region of cytoplasm
CCHDAGO:0070062extracellular exosome
CCTASGO:1904724tertiary granule lumen
CCTASGO:1904813ficolin-1-rich granule lumen
BPIEAGO:0001654eye development
BPIEAGO:0001666response to hypoxia
BPIEAGO:0006915apoptotic process
BPIDAGO:0006952defense response
BPIEAGO:0007420brain development
BPIEAGO:0007431salivary gland development
BPIEAGO:0007566embryo implantation
BPIEAGO:0008284positive regulation of cell proliferation
BPIEAGO:0009743response to carbohydrate
BPIDAGO:0010466negative regulation of peptidase activity
BPIEPGO:0010711negative regulation of collagen catabolic process
BPICGO:0010716negative regulation of extracellular matrix disassembly
BPIEPGO:0010716negative regulation of extracellular matrix disassembly
BPIEAGO:0031667response to nutrient levels
BPIEAGO:0032355response to estradiol
BPIEPGO:0034103regulation of tissue remodeling
BPIEAGO:0042747circadian sleep/wake cycle, REM sleep
BPIEAGO:0043067regulation of programmed cell death
BPTASGO:0043312neutrophil degranulation
BPTASGO:0043687post-translational protein modification
BPTASGO:0044267cellular protein metabolic process
BPIEAGO:0045740positive regulation of DNA replication
BPIDAGO:0045861negative regulation of proteolysis
BPIEAGO:0048678response to axon injury
BPIEAGO:0060009Sertoli cell development
BPIMPGO:0060311negative regulation of elastin catabolic process
BPIEPGO:0060313negative regulation of blood vessel remodeling
BPIEAGO:0060548negative regulation of cell death
BPIEAGO:0070301cellular response to hydrogen peroxide
BPIGIGO:0097435supramolecular fiber organization

Gene expression in normal tissue: CST3

Gene-model tissue-cancer distribution: Bubble Plot

Gene-drug pathway distribution

Pathways in CST3

DatabasePathway IDPathway Des.
reactomeR-HSA-168249Innate Immune System
reactomeR-HSA-168256Immune System
reactomeR-HSA-381426Regulation of Insulin-like Growth Factor (IGF) transport and uptake by Insulin-like Growth Factor Binding Proteins (IGFBPs)
reactomeR-HSA-392499Metabolism of proteins
reactomeR-HSA-597592Post-translational protein modification
reactomeR-HSA-6798695Neutrophil degranulation
reactomeR-HSA-8957275Post-translational protein phosphorylation
reactomeR-HSA-977225Amyloid fiber formation
kegghsa04970Salivary secretion - Homo sapiens (human)

Gene-Drug: Aster Plot

Drug IDDrug NameModel Num.

Gene in drug-gene network: Network Plot

Gene-drug targets distribution

Gene Structure: PDB

Models in CST3