Caveolins in rhabdomyosarcoma.
Bottom Line: Caveolins are scaffolding proteins that play a pivotal role in numerous processes, including caveolae biogenesis, vesicular transport, cholesterol homeostasis and regulation of signal transduction.There are three different isoforms (Cav-1, -2 and -3) that form homo- and hetero-aggregates at the plasma membrane and modulate the activity of a number of intracellular binding proteins.Cav-1 and Cav-3, in particular, are respectively expressed in the reserve elements (e.g. satellite cells) and in mature myofibres of skeletal muscle and their expression interplay characterizes the switch from muscle precursors to differentiated elements.
Affiliation: Department of Biomedical Sciences and Biotechnologies, Interuniversity Institute of Myology (IIM), University of Brescia, Brescia, Italy Department of Pathology, University of Brescia, Brescia, Italy.Show MeSH
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Mentions: Soft tissue sarcomas arise from primitive mesenchymal cells located throughout the body and make up to approximately 7% of all cancer cases in patients under the age of 20 [101, 102]. These tumours can be subdivided into two major groups: RMS and non-RMS soft tissue sarcomas, the latter including a miscellaneous of tumours, such as the synovial sarcoma, malignant fibrous histiocytoma, malignant peripheral nerve sheath tumour and fibrosarcoma . The immunohistochemical or molecular detection of myogenic regulatory factors, such as MyoD and myogenin [103–106], allows an RMS diagnosis, whereas the detection of myosin and other contractile proteins identifies more mature RMS phenotypes . The current classification of RMS into two major histological variants, termed embryonal (ERMS) and alveolar (ARMS), is supported by histopathological criteria and genetic signatures (Fig. 1).
Affiliation: Department of Biomedical Sciences and Biotechnologies, Interuniversity Institute of Myology (IIM), University of Brescia, Brescia, Italy Department of Pathology, University of Brescia, Brescia, Italy.