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Muscle-specific Gene Expression in Rhabdomyosarcomas and Stages of Human Fetal Skeletal Muscle Development

Muscle-specific Gene Expression in Rhabdomyosarcomas and Stages of Human Fetal Skeletal Muscle Development,Patricia N. Tonin,Heidi Scrable,Hiroyuki Sh

Muscle-specific Gene Expression in Rhabdomyosarcomas and Stages of Human Fetal Skeletal Muscle Development   (Citations: 33)
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Rhabdomyosarcomas (RMS) bear a morphological resemblance to developing striated muscle. It has been reported that two histologically distinct subtypes of RMS, embryonal and alveolar, behave differently in many clinical aspects, such as age distribution, primary site, and prog nosis. We have investigated the expression of various genes, which are preferentially expressed in normal muscle tissue or cell culture (actins, myosins, and creatine kinases, and myogenic regulatory genes MyoD, myogenin, MRF4, and My/5), in embryonal and alveolar subtypes and compared the results to the stages of developing human fetal limb muscle. The data showed that each of the RMS tumors tested, regardless of histológica! features, expressed MyoDl and MRF4 transcripts. Expres sion of the myogenin gene was detectable in all alveolar RMS (n = 8), whereas only 5 of 8 embryonal RMS expressed myogenin transcripts. Trace levels of MyfS transcripts were visible in all alveolar RMS and 7 of 8 embryonal RMS. The a-skeletal, «-cardiac,and 0- and -y-cytoplasmie actin transcripts were detectable in all alveolar RMS. While the ß- and 7-cytoplasmic actin transcripts were evident in all embryonal RMS, only 3 of 8 and 6 of 8 embryonal RMS expressed detectable levels of «- skeletal and «-cardiacactin transcripts, respectively. The embryonic form of myosin heavy chain was detectable in 1 of 8 of each type of tumor. Myosin light chain-1/3 transcripts were detectable in 4 of 8 alveolar RMS and 5 of 8 embryonal RMS. Brain creatine kinase transcripts were detectable in all alveolar RMS and 4 of 8 embryonal RMS, whereas none of the RMS samples contained detectable levels of the muscle form of creatine kinase. A comparison of the expression profiles with those of normal developing human fetal limb muscle (from 7.5 to 24 weeks' gestation) suggested that RMS resembled a relatively restricted segment of fetal muscle development. Furthermore, the data also showed a great deal of overlap in the differentiation state achieved by the embryonal and alveolar subtypes of RMS, suggesting that the clinicopathological differ ence between these two may not be due to malignant transformation of the cells from different positions in the normal pathway of myogenesis.
Published in 1991.
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    • ...Common among RMStumors is their myogenic phenotype, ranging from well-differentiated cells resembling embryonic muscle to variably undifferentiated round cells (2)...
    • ...Despite expressing MyoD and myogenin, key regulators of skeletal muscle determination and differentiation (13), ARMSshow limited structural evidence of muscle differentiation and seem to be in a primitive stage of skeletal myogenesis (2, 3). However, few clues exist as to the cellular genesis of ARMSbecause these tumors do not resemble known normal tissue and can arise at diverse anatomic sites not always associated with skeletal muscle...
    • ...PAX-FKHR–expressing human ARMSshare an expression profile with translocation-negative ARMSand ERMS(2, 3, 31), which includes the three aforementioned proteins...
    • ...This expression pattern recapitulates a similar pattern found in human ARMS(2, 12)...

    Yue-Xin Renet al. Mouse Mesenchymal Stem Cells Expressing PAX-FKHR Form Alveolar Rhabdom...

    • ...Investigators have proposed that these tumors are derived from primitive mesenchyme that retained its capacity for skeletal muscle differentiation (70, 71)...

    Jeffrey S. Chamberlainet al. Dystrophin-deficient mdx mice display a reduced life span and are susc...

    • ...[39, 44, 55, 57, 68, 85]) and in situ [45, 73, 84], without providing information at the protein level...
    • ...Previous biochemical studies have shown that fetal skeletal muscle of various species contains considerable amounts of CAA [44, 55, 57, 68, 73, 85, 89]...
    • ...[44, 55, 68, 73]), and expression of the CAA gene appears to precede that of the gene encoding skeletal muscle α-actin ([21, 85] and refs...
    • ...[45, 84]). Our present results extend these findings to the cellular level and allow the conclusion that at least until the second third of pregnancy, most human fetal skeletal muscle cells contain CAA in addition to skeletal muscle αactin (see also [44, 55, 57, 68, 73, 85, 89])...
    • ...That rhabdomyosarcomas, believed to be derived from relatively undifferentiated mesenchymal cells, synthesize CAA as the major sarcomeric actin isoform has previously been suggested on the basis of mRNA analyses [76, 85]...

    Roland Mollet al. The cardiac isoform of α-actin in regenerating and atrophic skeletal m...

    • ...2614 GENES & DEVELOPMENT 18:2614–2626 © 2004 by Cold Spring Harbor Laboratory Press ISSN 0890-9369/04; www.genesdev.org eages (Tonin et al. 1991)...
    • ...Similarly, the human rhabdomyosarcoma marker MyoD (Tonin et al. 1991) was uniformly expressed in the murine tumors (Fig. 5C)...

    Charles Kelleret al. Alveolar rhabdomyosarcomas in conditional Pax3:Fkhr mice: cooperativit...

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