Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0035412 (rhabdomyosarcoma)
 6,156 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

We performed immunoperoxidase studies in 29 cases of rhabdomyosarcoma from the Intergroup Rhabdomyosarcoma Study I using antisera against actin, myosin, myoglobin, alpha-actinin, and tropomyosin. Although each of these antisera reacted with some of the tumors, none reacted with all of the tumors, and some tumors showed no reactivity. Antimyosin reacted with more tumors than any of the others, while antiactin and antimyoglobin were about equally sensitive. Antitropomyosin and anti-alpha-actinin reacted with few of the tumors. The better-differentiated tumors were more likely to react compared with the poorly differentiated tumors.
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PMID:Immunohistochemical studies of rhabdomyosarcoma. 353 Jan 87

Most rhabdomyosarcomas are poorly differentiated malignant tumors. Dimethyl sulfoxide has been shown to modulate cell differentiation in cultured human cells. We induced differentiation in human rhabdomyosarcoma cell lines A-673, RD and A-204 with 1.25% dimethyl sulfoxide, and used desmin, the protein most frequently used as a marker of muscle cell differentiation, to trace this process. As alternative markers of the degree of differentiation, we quantified the expression of the proteins actin, tropomyosin and alpha-actinin in these cell lines, and followed the changes in expression of these proteins after induction for 8, 12, 24, 48 and 72 hrs. In the process of differentiation, protein expression in both the cytoplasm and cytoskeleton was significantly increased by treatments lasting 12 hrs. (alpha-actinin) and 24 hrs. (actin). On the basis of our results, alpha-actinin can be considered as an earlier marker of differentiation than actin in human rhabdomyosarcoma cell lines. However, the earliest indication of differentiation was a modification in desmin expression (8 hrs.). Because changes in tropomyosin expression were less marked, we consider this protein as a poor marker of rhabdomyosarcoma cell differentiation.
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PMID:Actin, tropomyosin and alpha-actinin as markers of differentiation in human rhabdomyosarcoma cell lines induced with dimethyl sulfoxide. 837 4

A pig rhabdomyosarcoma cell line (PRUM59) was established, and the immuno(histo)chemical and cytogenetic characterization of these cells was determined. At various swine farms in the Netherlands, pigs were observed that had solitary or multiple skin nodules, which were diagnosed as rhabdomyosarcomas. Cells of a tumor derived from a 3.5-week-old female pig were cultured for immunochemical and cytogenetic analyses. The cell line had characteristic features of undifferentiated muscle cells, similar to those observed in tumor tissue sections; they contained titin, a high-molecular weight protein specific for striated muscle, as dot-like aggregates and as filaments, desmin filaments and cross-striations, smooth muscle actin stress fibers, and vimentin filaments. The cells stained positively for striated muscle actin and tropomyosin as well. The immunohistochemical staining results were supported by results of immunoblotting experiments. Karyotyping of the cells revealed a deletion of a major part of Xq24-qter, a part of the long arm of 1 of the 2 X chromosomes. The other X chromosome and all autosomes appeared to be normal.
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PMID:Cultured pig rhabdomyosarcoma cells with a deletion of the Xq24-qter chromosome region: an immunochemical and cytogenetic characterization. 853 78

Gene transfection has been accomplished with a variety of techniques such as DEAE dextran, calcium phosphate coprecipitation, protoplast fusion, liposomes, microinjection and recombinant bacteriophages. However, transfection by electroporation, consisting of the reversible permeabilization of cell membranes after exposure to a pulsed electric field, has been shown to be the most rapid, simple and efficient method for the stable incorporation of genes in different cell lines. We studied rhabdomyosarcoma cells subjected to electroporation in two different vol. [400 microliters (group 1) and 150 microliters (group 2] of 140 mM NaCl/15 mM Hepes buffer, pH 7.2) and evaluated the effects of electroporation volume on growth and differentiation. Low sample volumes induced a terminal process of morphological and ultrastructural myogenic differentiation in rhabdomyosarcoma cells, which concluded with cell death. Our results suggest that in electroporation low sample vol. of rhabdomyosarcoma cells induced morphological and phenotypic differentiation, with increased expression of desmin, alpha-actinin and tropomyosin.
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PMID:Low sample volume causes differentiation in human rhabdomyosarcoma cell line RD subjected to electroporation. 899 25

Human embryonal cell line RD is derived from rhabdomyosarcoma, a tumor of childhood that arises from rhabdomyoblasts probably arrested somewhere along their pathway to maturation. Because actinomycin D is a drug of choice in the treatment of rhabdomyosarcomas, and because it has been used to induce differentiation as an alternative therapy for myeloproliferative syndromes, we treated RD cells with different concentrations of actinomycin D and evaluated the effects on growth and differentiation. Actinomycin D treatment in vitro caused time- and dose-dependent growth inhibition. Interestingly, RD cells treated with low doses (2.85 and 5.7 nmol/L) of actinomycin D for 6 days showed morphologic and phenotypic differentiation, with increased expression of desmin, alpha-actinin, and tropomyosin. However, treatment with 11.4 nmol/L actinomycin D strongly inhibited growth and had cytotoxic effects that prevented the cells from attaining myogenic differentiation. We conclude that exposure of this human embryonal rhabdomyosarcoma cell line to low concentrations of actinomycin D released the neoplastic cells from their blockade, allowing them to recover normal myogenic development. We suggest a potential role for differentiation therapy in the treatment of rhabdomyosarcomas.
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PMID:Actinomycin D treatment leads to differentiation and inhibits proliferation in rhabdomyosarcoma cells. 924 65

Convenient synthesis of 1-[[3-(2-hydroxyethylhetero)-1-alkoxy]alkyl]-5-fluorouracils 3-8 was accomplished via the use of tin (IV) chloride, capable of a 1,4-chelation on alkoxy-1,4-diheteroepanes. Increasing the reaction time led to 5-FU seven-membered nucleoside analogues which could be considered as upper isosteres of the effective antitumour agent Ftorafur. Using 1-[[3-(2-hydroxyethoxy)-1-alkoxy]propyl]-5-fluorouracil as a parent drug, several chemical modifications on the acyclic moiety were made with the aim of obtaining new compounds showing significant antiproliferative activity in rhabdomyosarcoma (RD) cells. 14 treatment in vitro caused time- and dose-dependent growth inhibition on RD cells. Interestingly, when they were treated with doses of 35 microM and 140 microM of 14 for 6 days, they showed morphological and phetotypic differentiation with increased expression of desmin, alpha-actinin and tropomyosin. We suggest a potential role for differentiation therapy as a therapeutic approach to the treatment of rhabdomyosarcoma.
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PMID:Diheterocyclanes as synthons for the preparation of novel series of nucleoside and acyclonucleoside analogues. 927 96

Rhabdomyosarcoma (RMS) is a highly malignant tumor that is histologically related to skeletal muscle, yet genetic and molecular lesions underlying its genesis and progression remain largely unknown. In this study we have compared the molecular profiles of two different mouse models of RMS, each associated with a defined primary genetic defect known to play a role in rhabdomyosarcomagenesis in man. We report that RMS of heterozygous Patched1 (Ptch1) mice show less aggressive growth and a greater degree of differentiation than RMS of heterozygous p53 mice. By means of cDNA microarray analysis we demonstrate that RMS in Ptch1 mutants predominantly express a number of myogenic markers, including myogenic differentiation 1, myosin heavy chain, actin, troponin and tropomyosin, as well as genes associated with Hedgehog/Patched signaling like insulin-like growth factor 2, forkhead box gene Foxf1 and the growth arrest and DNA-damage-inducible gene Gadd45a. In sharp contrast, RMS in p53 mutants display higher expression levels of cell cycle-associated genes like cyclin B1, cyclin-dependent kinase 4 and the proliferation marker Ki-67. These results demonstrate that different causative mutations lead to distinct gene expression profiles in RMS, which appear to reflect their different biological characteristics. Our results provide a first step towards a molecular classification of different forms of RMS. If the described differences can be confirmed in human RMS our results will contribute to a new molecular taxonomy of this cancer, which will be critical for gene mutation- and expression-specific therapy.
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PMID:Profiling the molecular difference between Patched- and p53-dependent rhabdomyosarcoma. 1548 Apr 23