Cst3

Summary

Gene Symbol: Cst3
Description: cystatin C
Alias: CysC, cystatin-C, cystatin-3
Species: mouse
Products:     Cst3

Top Publications

  1. Pawlik M, Sastre M, Calero M, Mathews P, Schmidt S, Nixon R, et al. Overexpression of human cystatin C in transgenic mice does not affect levels of endogenous brain amyloid Beta Peptide. J Mol Neurosci. 2004;22:13-8 pubmed
    b>Cystatin C, an inhibitor of cysteine proteases, colocalizes with amyloid beta (Abeta) in parenchymal and vascular amyloid deposits in brains of Alzheimer's disease (AD) patients, suggesting that cystatin C has a role in AD...
  2. Sukhova G, Wang B, Libby P, Pan J, Zhang Y, Grubb A, et al. Cystatin C deficiency increases elastic lamina degradation and aortic dilatation in apolipoprotein E-null mice. Circ Res. 2005;96:368-75 pubmed
    ..plaques have increased cysteine protease content and decreased levels of the endogenous inhibitor cystatin C, suggesting an imbalance that would favor matrix degradation in the arterial wall...
  3. Tizon B, Sahoo S, Yu H, Gauthier S, Kumar A, Mohan P, et al. Induction of autophagy by cystatin C: a mechanism that protects murine primary cortical neurons and neuronal cell lines. PLoS ONE. 2010;5:e9819 pubmed publisher
    b>Cystatin C (CysC) expression in the brain is elevated in human patients with epilepsy, in animal models of neurodegenerative conditions, and in response to injury, but whether up-regulated CysC expression is a manifestation of ..
  4. Bengtsson E, To F, Grubb A, Hakansson K, Wittgren L, Nilsson J, et al. Absence of the protease inhibitor cystatin C in inflammatory cells results in larger plaque area in plaque regression of apoE-deficient mice. Atherosclerosis. 2005;180:45-53 pubmed
    Matrix remodelling plays an important role in regulating plaque stability. Cystatin C, an inhibitor of the elastin-degrading cysteine proteases of the cathepsin family, is believed to be one of the key protease inhibitors in this process...
  5. Kaeser S, Herzig M, Coomaraswamy J, Kilger E, Selenica M, Winkler D, et al. Cystatin C modulates cerebral beta-amyloidosis. Nat Genet. 2007;39:1437-9 pubmed
    The CST3 Thr25 allele of CST3, which encodes cystatin C, leads to reduced cystatin C secretion and conveys susceptibility to Alzheimer's disease...
  6. Bengtsson E, To F, Hakansson K, Grubb A, Brånén L, Nilsson J, et al. Lack of the cysteine protease inhibitor cystatin C promotes atherosclerosis in apolipoprotein E-deficient mice. Arterioscler Thromb Vasc Biol. 2005;25:2151-6 pubmed
    ..b>Cystatin C, inhibitor of the collagen- and elastin-degrading cysteine proteases of the cathepsin family, is produced by ..
  7. Wang B, Sun J, Kitamoto S, Yang M, Grubb A, Chapman H, et al. Cathepsin S controls angiogenesis and tumor growth via matrix-derived angiogenic factors. J Biol Chem. 2006;281:6020-9 pubmed
    ..angiogenic islet formation and the growth of solid tumors, whereas the absence of its endogenous inhibitor cystatin C resulted in opposite phenotypes...
  8. Huh C, Hakansson K, Nathanson C, Thorgeirsson U, Jonsson N, Grubb A, et al. Decreased metastatic spread in mice homozygous for a null allele of the cystatin C protease inhibitor gene. Mol Pathol. 1999;52:332-40 pubmed
    ..To improve the current knowledge of the regulatory role of a major mammalian cysteine protease inhibitor, cystatin C, in such disease processes, a cystatin C deficient mouse was generated and characterized...
  9. Pirttilä T, Lukasiuk K, Hakansson K, Grubb A, Abrahamson M, Pitkanen A. Cystatin C modulates neurodegeneration and neurogenesis following status epilepticus in mouse. Neurobiol Dis. 2005;20:241-53 pubmed
    ..experiments were designed to study the cellular expression and functions of a cysteine proteinase inhibitor, cystatin C, whose gene expression is previously shown to be upregulated in the rat hippocampus during status epilepticus (..

More Information

Publications52

  1. Olsson T, Nygren J, Hakansson K, Lundblad C, Grubb A, Smith M, et al. Gene deletion of cystatin C aggravates brain damage following focal ischemia but mitigates the neuronal injury after global ischemia in the mouse. Neuroscience. 2004;128:65-71 pubmed
    b>Cystatin C is distributed in all human tissues and fluids with a particular abundance in the cerebrospinal fluid...
  2. Taupin P, Ray J, Fischer W, Suhr S, Hakansson K, Grubb A, et al. FGF-2-responsive neural stem cell proliferation requires CCg, a novel autocrine/paracrine cofactor. Neuron. 2000;28:385-97 pubmed
    ..This autocrine/paracrine cofactor is a glycosylated form of cystatin C (CCg), whose N-glycosylation is required for its activity...
  3. Kato T, Heike T, Okawa K, Haruyama M, Shiraishi K, Yoshimoto M, et al. A neurosphere-derived factor, cystatin C, supports differentiation of ES cells into neural stem cells. Proc Natl Acad Sci U S A. 2006;103:6019-24 pubmed
    ..by matrix-assisted laser desorption/ionization time-of-flight mass spectrometry, which turned out to be cystatin C. Purified cystatin C in place of the CMPNC could generate ES-NSCs efficiently with self-renewal and ..
  4. Sun B, Zhou Y, Halabisky B, Lo I, Cho S, Mueller Steiner S, et al. Cystatin C-cathepsin B axis regulates amyloid beta levels and associated neuronal deficits in an animal model of Alzheimer's disease. Neuron. 2008;60:247-57 pubmed publisher
    ..How Abeta-degrading enzymes are regulated remains largely unknown. Cystatin C (CysC, CST3) is an endogenous inhibitor of cysteine proteases, including cathepsin B (CatB), a recently ..
  5. Hu Y, Hung A, Cui H, Dawkins E, Bolos M, Foa L, et al. Role of cystatin C in amyloid precursor protein-induced proliferation of neural stem/progenitor cells. J Biol Chem. 2013;288:18853-62 pubmed publisher
    ..A secreted factor, identified as cystatin C, was found to be responsible for this effect...
  6. Tominaga Y, Tamguney T, Kolesnichenko M, Bilanges B, Stokoe D. Translational deregulation in PDK-1-/- embryonic stem cells. Mol Cell Biol. 2005;25:8465-75 pubmed
    ..We show that PKB activity is a critical downstream component of PDK-1 in mediating translation of cystatin C, RANKL, and Rab11a, whereas mTOR activity is less important for effective translation of these targets.
  7. Pan H, Wang D, Zhang X, Zhou D, Zhang H, Qian Q, et al. Amyloid ? Is Not the Major Factor Accounting for Impaired Adult Hippocampal Neurogenesis in Mice Overexpressing Amyloid Precursor Protein. Stem Cell Reports. 2016;7:707-718 pubmed publisher
    ..Our results suggested that hAPP was more likely an important factor inhibiting adult neurogenesis, and A? was not the major factor affecting neurogenesis in the adult hippocampus of hAPP mice. ..
  8. Kaseda R, Iino N, Hosojima M, Takeda T, Hosaka K, Kobayashi A, et al. Megalin-mediated endocytosis of cystatin C in proximal tubule cells. Biochem Biophys Res Commun. 2007;357:1130-4 pubmed
    Serum levels of cystatin C, an endogenous cysteine proteinase inhibitor, are often used as an indicator of glomerular filtration rate...
  9. Huh C, Nagle J, Kozak C, Abrahamson M, Karlsson S. Structural organization, expression and chromosomal mapping of the mouse cystatin-C-encoding gene (Cst3). Gene. 1995;152:221-6 pubmed
    b>Cystatin C (CstC) is a potent cysteine-proteinase inhibitor. The structure of the mouse CstC-encoding gene (Cst3) was examined by sequencing a 6.1-kb genomic DNA containing the entire gene, as well as 0.9 kb of 5' flanking and 1...
  10. Kanno Y, Tamura M, Chuma S, Sakura T, Machida T, Nakatsuji N. A cystatin-related gene, testatin/cresp, shows male-specific expression in germ and somatic cells from the initial stage of murine gonadal sex-differentiation. Int J Dev Biol. 1999;43:777-84 pubmed
  11. Afonso S, Tovar C, Romagnano L, Babiarz B. Control and expression of cystatin C by mouse decidual cultures. Mol Reprod Dev. 2002;61:155-63 pubmed
    ..Our work has focused on cystatin C, the secreted inhibitor of cathepsins B and L...
  12. Tu G, Aldred A, Southwell B, Schreiber G. Strong conservation of the expression of cystatin C gene in choroid plexus. Am J Physiol. 1992;263:R195-200 pubmed
    The expression of the cystatin C gene was studied by Northern analysis of RNA isolated from the choroid plexus, other brain tissues, and liver from 11 mammalian and 4 avian species...
  13. Danjo A, Yamaza T, Kido M, Shimohira D, Tsukuba T, Kagiya T, et al. Cystatin C stimulates the differentiation of mouse osteoblastic cells and bone formation. Biochem Biophys Res Commun. 2007;360:199-204 pubmed
    b>Cystatin C (CysC) is a natural cysteine proteinase inhibitor that suppresses the differentiation and bone-resorptive function of osteoclasts...
  14. Tohonen V, Osterlund C, Nordqvist K. Testatin: a cystatin-related gene expressed during early testis development. Proc Natl Acad Sci U S A. 1998;95:14208-13 pubmed
    ..This gene therefore represents one of the putative downstream targets likely to have an essential role in tissue reorganization during early testis development. ..
  15. Schulte S, Sun J, Libby P, MacFarlane L, Sun C, Lopez Ilasaca M, et al. Cystatin C deficiency promotes inflammation in angiotensin II-induced abdominal aortic aneurisms in atherosclerotic mice. Am J Pathol. 2010;177:456-63 pubmed publisher
    An imbalance between cysteinyl cathepsins and their principal endogenous inhibitor cystatin C (CystC) may favor proteolysis in the pathogenesis of human abdominal aortic aneurysms (AAA), yet a direct role of CystC in AAA remains unproven...
  16. CARLSON E, Magid R, Petryk A, Georgieff M. Iron deficiency alters expression of genes implicated in Alzheimer disease pathogenesis. Brain Res. 2008;1237:75-83 pubmed publisher
    ..Quantitative PCR confirmed altered regulation in 6 of 7 Alzheimer-related genes (Apbb1, C1qa, Clu, App, Cst3, Fn1, Htatip) in iron-deficient rats relative to iron-sufficient controls at P15...
  17. Ghidoni R, Paterlini A, Albertini V, Glionna M, Monti E, Schiaffonati L, et al. Cystatin C is released in association with exosomes: a new tool of neuronal communication which is unbalanced in Alzheimer's disease. Neurobiol Aging. 2011;32:1435-42 pubmed publisher
    ..Multiple lines of research support a neuroprotective role for cystatin C in Alzheimer's disease (AD)...
  18. Solem M, Rawson C, Lindburg K, Barnes D. Transforming growth factor beta regulates cystatin C in serum-free mouse embryo (SFME) cells. Biochem Biophys Res Commun. 1990;172:945-51 pubmed
    ..serum-free mouse embryo (astrocyte precursor) cells isolated a strongly TGF beta-regulated mRNA that codes for cystatin C, a cysteine protease inhibitor...
  19. Li W, Sultana N, Siraj N, Ward L, Pawlik M, Levy E, et al. Autophagy dysfunction and regulatory cystatin C in macrophage death of atherosclerosis. J Cell Mol Med. 2016;20:1664-72 pubmed publisher
    ..Expression of cystatin C (CysC) is decreased in human atheroma, and CysC deficiency enhances atherosclerosis in mice...
  20. Watanabe S, Hayakawa T, Wakasugi K, Yamanaka K. Cystatin C protects neuronal cells against mutant copper-zinc superoxide dismutase-mediated toxicity. Cell Death Dis. 2014;5:e1497 pubmed publisher
    ..b>Cystatin C (CysC), an endogenous cysteine protease inhibitor, is a major protein component of Bunina bodies observed in the ..
  21. Whelly S, Serobian G, Borchardt C, Powell J, Johnson S, Hakansson K, et al. Fertility defects in mice expressing the L68Q variant of human cystatin C: a role for amyloid in male infertility. J Biol Chem. 2014;289:7718-29 pubmed publisher
    Hereditary cystatin C amyloid angiopathy is an autosomal dominant disorder in which a variant form of cystatin C (L68Q) readily forms amyloid deposits in cerebral arteries in affected individuals resulting in early death...
  22. Yu W, Liu J, Shi M, Wang J, Xiang M, Kitamoto S, et al. Cystatin C deficiency promotes epidermal dysplasia in K14-HPV16 transgenic mice. PLoS ONE. 2010;5:e13973 pubmed publisher
    ..of cysteine protease cathepsin S, but concomitantly reduced expression of cathepsin endogenous inhibitor cystatin C in the skin tissue extract...
  23. Kaur G, Mohan P, Pawlik M, DeRosa S, Fajiculay J, Che S, et al. Cystatin C rescues degenerating neurons in a cystatin B-knockout mouse model of progressive myoclonus epilepsy. Am J Pathol. 2010;177:2256-67 pubmed publisher
    In vitro studies have shown that cystatin C (CysC) is neuroprotective. Here we demonstrate that CysC is neuroprotective in vivo, in a mouse model of the inherited neurodegenerative disorder, progressive myoclonic epilepsy type 1 (EPM1)...
  24. Xie L, Terrand J, Xu B, Tsaprailis G, Boyer J, Chen Q. Cystatin C increases in cardiac injury: a role in extracellular matrix protein modulation. Cardiovasc Res. 2010;87:628-35 pubmed publisher
    ..Comparison between the two cell types leads to the finding that H(2)O(2) caused an elevated cystatin C protein in the conditioned medium from cardiomyocytes...
  25. Yamaza T, Tsuji Y, Goto T, Kido M, Nishijima K, Moroi R, et al. Comparison in localization between cystatin C and cathepsin K in osteoclasts and other cells in mouse tibia epiphysis by immunolight and immunoelectron microscopy. Bone. 2001;29:42-53 pubmed
    We compared the distribution of a cysteine proteinase inhibitor, cystatin C, with that of cathepsin K in osteoclasts of the mouse tibia by immunolight and immunoelectron microscopy...
  26. Ying G, Huang C, Jiang Z, Liu X, Jing N, Zhou C. Up-regulation of cystatin C expression in the murine hippocampus following perforant path transections. Neuroscience. 2002;112:289-98 pubmed
    ..Here we report the expression of cystatin C, a major cysteine protease inhibitor of mammalian animals, in the murine hippocampus at 3, 7, 15 and 30 days ..
  27. Kumada T, Hasegawa A, Iwasaki Y, Baba H, Ikenaka K. Isolation of cystatin C via functional cloning of astrocyte differentiation factors. Dev Neurosci. 2004;26:68-76 pubmed
    ..One cDNA clone that repeatedly upregulated lacZ expression encoded cystatin C (CysC), a cysteine protease inhibitor...
  28. Baston Buest D, Schanz A, Buest S, Fischer J, Kruessel J, Hess A. The embryo's cystatin C and F expression functions as a protective mechanism against the maternal proteinase cathepsin S in mice. Reproduction. 2010;139:741-8 pubmed publisher
    ..Additionally, the observed decrease in CD3(+) cells leads to the hypothesis that cells of the cytotoxic T-cell group are down-regulated in the decidua to support the implantation and ensure the survival of the embryo. ..
  29. Hakansson K, Huh C, Grubb A, Karlsson S, Abrahamson M. Mouse and rat cystatin C: Escherichia coli production, characterization and tissue distribution. Comp Biochem Physiol B Biochem Mol Biol. 1996;114:303-11 pubmed
    Recombinant mouse (Mus musculus) and rat (Rattus norvegicus) cystatin C were produced by expression in Escherichia coli, isolated and functionally characterized. The mouse and rat inhibitors were both fully active in titrations of papain...
  30. Kopitz C, Gerg M, Gansbacher B, Krüger A. Plasminogen activator inhibitor-2, but not cystatin C, inhibits the prometastatic activity of tissue inhibitor of metalloproteinases-1 in the liver. Hum Gene Ther. 2008;19:1039-49 pubmed publisher
    ..of TIMP-1-promoted experimental liver metastases of lacZ-tagged human fibrosarcoma cells by overexpression of cystatin C, a natural inhibitor of CPs, in the murine host...
  31. Frygelius J, Oscarson M, Nordqvist K, Wedell A, Tohonen V. The reproductive tissue specific cystatin subgroup of genes: expression during gonadal development in wildtype and testatin knockout animals. Sex Dev. 2007;1:363-72 pubmed publisher
    ..The most likely candidate/s would be CstSC, CstTE-1/Cres3 or Cres as they are expressed in the fetal testicular tubules in early testis differentiation together with testatin. ..
  32. Cornwall G, Hsia N, Sutton H. Structure, alternative splicing and chromosomal localization of the cystatin-related epididymal spermatogenic gene. Biochem J. 1999;340 ( Pt 1):85-93 pubmed
    ..of the cystatin family 2 genes, and the Cres exon/intron splice junctions occur in identical locations as in the cystatin C gene...
  33. Smart N, Hill A, Cross J, Riley P. A differential screen for putative targets of the bHLH transcription factor Hand1 in cardiac morphogenesis. Mech Dev. 2002;119 Suppl 1:S65-71 pubmed
    ..Thymosin beta4 was found to be down-regulated whereas cystatin C and alphaCA were up-regulated in Hand1-null embryoid bodies...
  34. Xu Y, Lindemann P, Vega Ramos J, Zhang J, Villadangos J. Developmental regulation of synthesis and dimerization of the amyloidogenic protease inhibitor cystatin C in the hematopoietic system. J Biol Chem. 2014;289:9730-40 pubmed publisher
    The cysteine protease inhibitor cystatin C is thought to be secreted by most cells and eliminated in the kidneys, so its concentration in plasma is diagnostic of kidney function...
  35. Bäcklund A, Holmdahl M, Mattsson R, Hakansson K, Lindström V, Nandakumar K, et al. Cystatin C influences the autoimmune but not inflammatory response to cartilage type II collagen leading to chronic arthritis development. Arthritis Res Ther. 2011;13:R54 pubmed publisher
    ..A natural inhibitor of cysteine proteases is cystatin C. Cystatin C-deficient, sufficient and heterozygous mice were tested for onset, incidence and severity of CIA...
  36. El Sukkari D, Wilson N, Hakansson K, Steptoe R, Grubb A, Shortman K, et al. The protease inhibitor cystatin C is differentially expressed among dendritic cell populations, but does not control antigen presentation. J Immunol. 2003;171:5003-11 pubmed
    ..It has been proposed that the cysteine protease inhibitor cystatin C (CyC) plays a pivotal role in the control of this process by regulating the activity of cathepsin S, a protease ..
  37. Ji X, Yao L, Wang M, Liu X, Peng S, Li K, et al. Cystatin C attenuates insulin signaling transduction by promoting endoplasmic reticulum stress in hepatocytes. FEBS Lett. 2015;589:3938-44 pubmed publisher
    It has been reported that cystatin c (Cys C) closely correlates with metabolic disorders such as obesity and diabetes. However, it is still unknown whether Cys C plays a role for these disorders...
  38. de Sousa Pereira P, Abrantes J, Pinheiro A, Colaço B, Vitorino R, Esteves P. Evolution of C, D and S-type cystatins in mammals: an extensive gene duplication in primates. PLoS ONE. 2014;9:e109050 pubmed publisher
    ..a family of inhibitors of cysteine peptidases that comprises the salivary cystatins (D and S-type cystatins) and cystatin C. These cystatins are encoded by a multigene family (CST3, CST5, CST4, CST1 and CST2) organized in tandem in the ..
  39. Suzuki Y, Jin C, Yazawa I. Cystatin C triggers neuronal degeneration in a model of multiple system atrophy. Am J Pathol. 2014;184:790-9 pubmed publisher
    ..In this study, we identified cystatin C, an oligodendrocyte-derived secretory protein that triggers ?-syn up-regulation and insoluble ?-syn accumulation,..
  40. Frendéus K, Wallin H, Janciauskiene S, Abrahamson M. Macrophage responses to interferon-gamma are dependent on cystatin C levels. Int J Biochem Cell Biol. 2009;41:2262-9 pubmed publisher
    The aim of the present investigation was to elucidate possible effects of cystatin C on inflammatory responses mediated by macrophages...
  41. Xu Y, Schnorrer P, Proietto A, Kowalski G, Febbraio M, Acha Orbea H, et al. IL-10 controls cystatin C synthesis and blood concentration in response to inflammation through regulation of IFN regulatory factor 8 expression. J Immunol. 2011;186:3666-73 pubmed publisher
    b>Cystatin C (CstC) is a cysteine protease inhibitor of major clinical importance. Low concentration of serum CstC is linked to atherosclerosis...
  42. Ma J, Tanaka K, Yamada G, Ikenaka K. Induced expression of cathepsins and cystatin C in a murine model of demyelination. Neurochem Res. 2007;32:311-20 pubmed
    ..Analysis revealed upregulated expression of cathepsins L, H and B and their inhibitor, cystatin C. By in situ hybridization, the induction of cathepsins was primarily limited to microglia/macrophages of the ..
  43. Afonso S, Romagnano L, Babiarz B. The expression and function of cystatin C and cathepsin B and cathepsin L during mouse embryo implantation and placentation. Development. 1997;124:3415-25 pubmed
    ..We focused on the cysteine proteinases, cathepsins B and L, and their inhibitor cystatin C. Northern blots showed that trophoblast expressed cathepsin B throughout the invasive period (days 5.5-10.5)...