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Gene Review

Cst3  -  cystatin C

Mus musculus

Synonyms: CysC, Cystatin-3, Cystatin-C
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Disease relevance of Cst3


Psychiatry related information on Cst3

  • Cystatin C has also amyloidogenic properties and is co-localized with beta-amyloid in degenerated neurons in Alzheimer's disease, suggesting a role in neuronal degeneration [2].

High impact information on Cst3


Chemical compound and disease context of Cst3

  • A cystatin C variant with L68Q substitution and a truncation of 10 NH2-terminal residues is the major constituent of the amyloid deposited in the cerebral vasculature of patients with the Icelandic form of hereditary cerebral hemorrhage with amyloidosis (HCHWA-I) [7].
  • In contrast to wild-type cystatin C, recombinant human cystatin C with Gly substitutions for residues Arg(8), Leu(9), Val(10), and Trp(106), and with low or nonexistent affinity for cysteine proteinases, did not display any inhibitory effect on bone resorption [8].
  • Z-RLVG-CHN(2), in which the reactive carboxyterminal diazomethane was substituted by nonreactive groups [-OH, -NH(2), or -N(CH(3))(2)], resulted in peptidyl derivatives, which, in contrast to Z-RLVG-CHN(2) and cystatin C, inhibited neither cysteine proteinases nor bone resorption [8].
  • Ukrain treatment of LS lymphosarcoma was also followed by increased levels of cystatin C in tumor tissue (4-fold); cyclophosphane plus Ukrain had a similar positive effect [9].
  • Peritoneal macrophages from mice with experimental HA-1 hepatoma compared to those from intact mice secreted more cystatin C with maximum polysaccharide-stimulated secretion after 30 min of incubation [10].

Biological context of Cst3


Anatomical context of Cst3


Associations of Cst3 with chemical compounds


Regulatory relationships of Cst3


Other interactions of Cst3


Analytical, diagnostic and therapeutic context of Cst3

  • The mouse Cst3 mRNA was detected in all of thirteen tissues examined by Northern blot analysis [11].
  • Apolipoprotein E-deficient mice (apoE-/-) were given a Western-type diet 15 weeks prior transplantation with bone marrow from mice lacking cystatin C (cysC-/-) or cystatin C positive (cysC+/+) mice, in both cases apoE+/+ to create conditions favouring plaque regression [13].
  • After 25 weeks of atherogenic diet, mice lacking apoE and cystatin C (cysC(-/-) apoE(-/-)) had larger subvalvular plaques compared with cysC(+/+) apoE(-/-) mice (766,000+/-20,000 microm2 per section versus 662,000+/-19,000 microm2 per section; P=0.001), suggesting an atheroprotective role of cystatin C [18].
  • Northern and western blotting showed that cystatin C, the main inhibitor of cathepsins, was a major product of the decidualizing stroma [12].
  • To explore possible neuropathological consequences of this genetic association, we examined expression of cystatin C in brains from 22 AD and 11 control patients by immunohistochemistry [25].


  1. Regulation of cysteine cathepsin expression by oxidative stress in the retinal pigment epithelium/choroid of the mouse. Alizadeh, P., Smit-McBride, Z., Oltjen, S.L., Hjelmeland, L.M. Exp. Eye Res. (2006) [Pubmed]
  2. Gene deletion of cystatin C aggravates brain damage following focal ischemia but mitigates the neuronal injury after global ischemia in the mouse. Olsson, T., Nygren, J., Håkansson, K., Lundblad, C., Grubb, A., Smith, M.L., Wieloch, T. Neuroscience (2004) [Pubmed]
  3. Cystatin C in LS lymphosarcoma and HA-1 hepatoma treated with Ukrain and cyclophosphamide and involvement of apoptosis. Korolenko, T.A., Poteryaeva, O.N., Djanayeva, S.J., Svechnikova, I.G., Kaledin, V.I., Timofeyeva, O.A., Filipenko, M.L., Nowicky, J. Drugs under experimental and clinical research. (2000) [Pubmed]
  4. Developmental regulation of invariant chain proteolysis controls MHC class II trafficking in mouse dendritic cells. Pierre, P., Mellman, I. Cell (1998) [Pubmed]
  5. Bacterial growth blocked by a synthetic peptide based on the structure of a human proteinase inhibitor. Björck, L., Akesson, P., Bohus, M., Trojnar, J., Abrahamson, M., Olafsson, I., Grubb, A. Nature (1989) [Pubmed]
  6. Constitutive secretion of cystatin C (gamma-trace) by monocytes and macrophages and its downregulation after stimulation. Warfel, A.H., Zucker-Franklin, D., Frangione, B., Ghiso, J. J. Exp. Med. (1987) [Pubmed]
  7. Instability of the amyloidogenic cystatin C variant of hereditary cerebral hemorrhage with amyloidosis, Icelandic type. Wei, L., Berman, Y., Castaño, E.M., Cadene, M., Beavis, R.C., Devi, L., Levy, E. J. Biol. Chem. (1998) [Pubmed]
  8. A peptidyl derivative structurally based on the inhibitory center of cystatin C inhibits bone resorption in vitro. Johansson, L., Grubb, A., Abrahamson, M., Kasprzykowski, F., Kasprzykowska, R., Grzonka, Z., Lerner, U.H. Bone (2000) [Pubmed]
  9. Cysteine proteinase inhibitor level in tumor and normal tissues in control and cured mice. Poteryaeva, O.N., Falameyeva, O.V., Korolenko, T.A., Kaledin, V.I., Djanayeva, S.J., Nowicky, J.W., Sandula, J. Drugs under experimental and clinical research. (2000) [Pubmed]
  10. Effect of polysaccharides and human plasma lipoproteins on the secretion of cystatin C by peritoneal macrophages from normal and tumor bearing mice. Poteryaeva, O.N., Polyakov, L.M., Korolenko, T.A., Zueva, T.V., Russkikh, G.S., Panin, L.E. Biochemistry Mosc. (2004) [Pubmed]
  11. Structural organization, expression and chromosomal mapping of the mouse cystatin-C-encoding gene (Cst3). Huh, C., Nagle, J.W., Kozak, C.A., Abrahamson, M., Karlsson, S. Gene (1995) [Pubmed]
  12. The expression and function of cystatin C and cathepsin B and cathepsin L during mouse embryo implantation and placentation. Afonso, S., Romagnano, L., Babiarz, B. Development (1997) [Pubmed]
  13. Absence of the protease inhibitor cystatin C in inflammatory cells results in larger plaque area in plaque regression of apoE-deficient mice. Bengtsson, E., To, F., Grubb, A., Håkansson, K., Wittgren, L., Nilsson, J., Jovinge, S. Atherosclerosis (2005) [Pubmed]
  14. Isolation of cystatin C via functional cloning of astrocyte differentiation factors. Kumada, T., Hasegawa, A., Iwasaki, Y., Baba, H., Ikenaka, K. Dev. Neurosci. (2004) [Pubmed]
  15. Cystatin C deficiency increases elastic lamina degradation and aortic dilatation in apolipoprotein E-null mice. Sukhova, G.K., Wang, B., Libby, P., Pan, J.H., Zhang, Y., Grubb, A., Fang, K., Chapman, H.A., Shi, G.P. Circ. Res. (2005) [Pubmed]
  16. The cystatin-related epididymal spermatogenic protein inhibits the serine protease prohormone convertase 2. Cornwall, G.A., Cameron, A., Lindberg, I., Hardy, D.M., Cormier, N., Hsia, N. Endocrinology (2003) [Pubmed]
  17. Progesterone and interferon-tau regulate cystatin C in the endometrium. Song, G., Spencer, T.E., Bazer, F.W. Endocrinology (2006) [Pubmed]
  18. Lack of the cysteine protease inhibitor cystatin C promotes atherosclerosis in apolipoprotein E-deficient mice. Bengtsson, E., To, F., Håkansson, K., Grubb, A., Brånén, L., Nilsson, J., Jovinge, S. Arterioscler. Thromb. Vasc. Biol. (2005) [Pubmed]
  19. A differential screen for putative targets of the bHLH transcription factor Hand1 in cardiac morphogenesis. Smart, N., Hill, A.A., Cross, J.C., Riley, P.R. Mech. Dev. (2002) [Pubmed]
  20. The protease inhibitor cystatin C is differentially expressed among dendritic cell populations, but does not control antigen presentation. El-Sukkari, D., Wilson, N.S., Hakansson, K., Steptoe, R.J., Grubb, A., Shortman, K., Villadangos, J.A. J. Immunol. (2003) [Pubmed]
  21. Transforming growth factor beta regulates cystatin C in serum-free mouse embryo (SFME) cells. Solem, M., Rawson, C., Lindburg, K., Barnes, D. Biochem. Biophys. Res. Commun. (1990) [Pubmed]
  22. Increased cystatin C in astrocytes of transgenic mice expressing the K670N-M671L mutation of the amyloid precursor protein and deposition in brain amyloid plaques. Steinhoff, T., Moritz, E., Wollmer, M.A., Mohajeri, M.H., Kins, S., Nitsch, R.M. Neurobiol. Dis. (2001) [Pubmed]
  23. Presenilin 2 mutations alter cystatin C trafficking in mouse primary neurons. Ghidoni, R., Benussi, L., Paterlini, A., Missale, C., Usardi, A., Rossi, R., Barbiero, L., Spano, P., Binetti, G. Neurobiol. Aging (2007) [Pubmed]
  24. Differential localization of cysteine protease inhibitors and a target cysteine protease, cathepsin B, by immuno-confocal microscopy. Calkins, C.C., Sameni, M., Koblinski, J., Sloane, B.F., Moin, K. J. Histochem. Cytochem. (1998) [Pubmed]
  25. Elevation of cystatin C in susceptible neurons in Alzheimer's disease. Deng, A., Irizarry, M.C., Nitsch, R.M., Growdon, J.H., Rebeck, G.W. Am. J. Pathol. (2001) [Pubmed]
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