Abstract
Mutations in the C-terminal domain of tissue inhibitor of metalloproteinases-3 (TIMP-3) are linked with Sorsby fundus dystrophy (autosomal dominant retinal dystrophy). Experiments on timp-3 gene knock-out and knock-in mice, carriers of dystrophy-linked Timp-3S156C mutation, showed that expression of mutant Timp-3S156C, in contrast to the normal one, is not regulated by phorbol-myristate-12-acetate and is retained at a high level in a medium without FCS. The mutant protein is not transported into the nucleus, while fibroblasts expressing it survive without FCS and other growth factors. It is hypothesized that normal Timp-3 is involved in the regulation of expression of its own timp-3 gene and, probably, other genes associated with growth and proliferation. The absence of this characteristic in mutant Timp-3S156C is presumably responsible for its accumulation in the extracellular matrix.
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Translated from Byulleten’ Eksperimental’noi Biologii i Meditsiny, Vol. 143, No. 1, pp. 70–73, January, 2007
This Incomplete work had been published without approval of the team where the work took place. Thus this article was retracted by the editors. The retraction note to this article is available at http://dx.doi.org/10.1007/s10517-009-0586-z.
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Sukhikh, G.T., Soboleva, G.M. RETRACTED ARTICLE: Sorsby fundus dystrophy-related mutation in tissue inhibitor of metalloproteinases-3 impairs regulation of its expression in mouse fibroblasts. Bull Exp Biol Med 143, 64–67 (2007). https://doi.org/10.1007/s10517-007-0018-x
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DOI: https://doi.org/10.1007/s10517-007-0018-x