Rhabdomyosarcoma – working out the pathways

@article{Merlino1999RhabdomyosarcomaW,
  title={Rhabdomyosarcoma – working out the pathways},
  author={Glenn Merlino and Lee J. Helman},
  journal={Oncogene},
  year={1999},
  volume={18},
  pages={5340-5348}
}
Rhabdomyosarcomas constitute a collection of childhood malignancies thought to arise as a consequence of regulatory disruption of skeletal muscle progenitor cell growth and differentiation. Our understanding of the pathogenesis of this neoplasm has recently benefited from the study of normal and malignant myogenic cells in vitro, facilitating the identification of diagnostic cytogenetic markers and the elucidation of mechanisms by which myogenesis is regulated. It is now appreciated that the… 
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248 Citations

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The data suggests that RMS represent an arrested state of development balanced between myoblast and myotube, and that manipulation of components of the myogenic gene program can 'tip the balance' and restore their ability to differentiate.
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Compared mouse models of rhabdomyosarcoma show less aggressive growth and a greater degree of differentiation than RMS of heterozygous p53 mice, and it is demonstrated that different causative mutations lead to distinct gene expression profiles in RMS, which appear to reflect their different biological characteristics.
Myostatin inhibits rhabdomyosarcoma cell proliferation through an Rb-independent pathway
TLDR
The analysis of Rb reveals that there was no change in its phosphorylation status with myostatin treatment, consistent with D-type-cyclin–Cdk4/6 complexes being active in the absence of p21, and suggests that mystatin could potentially be used as an inhibitor of RMS proliferation.
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It is demonstrated that in vitro differentiation of alveolar RMS cells decreases their metastatic behavior by reducing both the expression of the MET and CXCR4 receptors and their migratory response to HGF and SDF-1.
Genetic modeling of Ras-induced human rhabdomyosarcoma.
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It is shown that activation of the HER-2/neu oncogene coupled with inactivation of the oncosuppressor gene p53 causes rhabdomyosarcoma in mice, leading to the hypothesis that the interaction between HER family genes and the p53 pathway might be involved in the origin of human rhabDomyosARcoma.
Molecular characterization of Patched‐associated rhabdomyosarcoma
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The data suggest that the formation of RMSs in Ptch1 mutants is associated with the ability of tumour cells to resist apoptosis.
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