The cellular response to ectopic overexpression of the tuberous sclerosis genes, TSC1 and TSC2: a proteomic approach

  • Authors:
    • Markus Hengstschläger
    • Margit Rosner
    • Michael Fountoulakis
    • Gert Lubec
  • View Affiliations

  • Published online on: September 1, 2005     https://doi.org/10.3892/ijo.27.3.831
  • Pages: 831-838
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Abstract

The genes, TSC1 on chromosome 9q34, encoding hamartin, and TSC2 on chromosome 16p13.3, encoding tuberin, are responsible for tuberous sclerosis (TSC). TSC is an autosomal dominant tumor suppressor gene syndrome affecting about 1 in 6000 individuals. It is characterized by mental retardation and epilepsy. A variety of tumors characteristically occur in different organs of TSC patients and are believed to result from defects in cell cycle/cell size control. Hamartin and tuberin form a complex providing a tentative explanation for the similar disease phenotype in TSC patients with mutations in either of these genes. Beside overlap in many features of patients with TSC1 and TSC2 mutations, data accumulated providing evidence for specific clinical differences. In this study, we performed a proteomic approach of two-dimensional gel electrophoresis with subsequent mass spectrometrical identification of protein spots after ectopic overexpression of human TSC1 or TSC2. We found the protein levels of the calumenin precursor; the complement component 1; heterogeneous nuclear ribonucleoproteins, C1/C2; heterogeneous nuclear ribonucleoprotein, C1-like protein; nascent polypeptide-associated complex-α; proteasome subunit α type 5; reticulocalbin 1 precursor; translationally-controlled tumor protein; UV excision repair protein, RAD23 homolog B; elongation factor 1-δ; and the eukaryotic initiation factors, eIF-4A-like NUK-34 and eIF-6; to be deregulated upon ectopic TSC gene expression. These findings suggest that deregulation of the control of these new target proteins might contribute to the development of tubers/hamartomas in tuberous sclerosis patients. The data are presented and discussed in the context of the published literature on proteomic approaches for the identification of targets of the TSC genes.

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September 2005
Volume 27 Issue 3

Print ISSN: 1019-6439
Online ISSN:1791-2423

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Spandidos Publications style
Hengstschläger M, Rosner M, Fountoulakis M and Lubec G: The cellular response to ectopic overexpression of the tuberous sclerosis genes, TSC1 and TSC2: a proteomic approach. Int J Oncol 27: 831-838, 2005
APA
Hengstschläger, M., Rosner, M., Fountoulakis, M., & Lubec, G. (2005). The cellular response to ectopic overexpression of the tuberous sclerosis genes, TSC1 and TSC2: a proteomic approach. International Journal of Oncology, 27, 831-838. https://doi.org/10.3892/ijo.27.3.831
MLA
Hengstschläger, M., Rosner, M., Fountoulakis, M., Lubec, G."The cellular response to ectopic overexpression of the tuberous sclerosis genes, TSC1 and TSC2: a proteomic approach". International Journal of Oncology 27.3 (2005): 831-838.
Chicago
Hengstschläger, M., Rosner, M., Fountoulakis, M., Lubec, G."The cellular response to ectopic overexpression of the tuberous sclerosis genes, TSC1 and TSC2: a proteomic approach". International Journal of Oncology 27, no. 3 (2005): 831-838. https://doi.org/10.3892/ijo.27.3.831