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Rhabdomyosarcoma and Wilms tumors contain a subpopulation of noggin producing, myogenic cells immunoreactive for lens beaded filament proteins
Journal article   Open access   Peer reviewed

Rhabdomyosarcoma and Wilms tumors contain a subpopulation of noggin producing, myogenic cells immunoreactive for lens beaded filament proteins

Jacquelyn Gerhart, Kathryn Behling, Michele Paessler, LaBraya Milton, Gregory Bramblett, Denise Garcia, Meghan Pitts, Reginald Hurtt, Mitchell Crawford, Richard Lackman, …
PloS one, v 14(4), e0214758
2019
DOI: https://doi.org/10.1371/journal.pone.0214758
PMID: 30973903
Featured in Collection :   UN Sustainable Development Goals @ Drexel
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https://doi.org/10.1371/journal.pone.0214758View
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Abstract

Animals Antibodies, Monoclonal - immunology Carrier Proteins - immunology Carrier Proteins - metabolism Cell Line Eye Proteins - genetics Eye Proteins - immunology Eye Proteins - metabolism Humans Intermediate Filament Proteins - genetics Intermediate Filament Proteins - immunology Intermediate Filament Proteins - metabolism Lens, Crystalline - cytology Lens, Crystalline - metabolism Mice Microscopy, Fluorescence MyoD Protein - immunology MyoD Protein - metabolism Rhabdomyosarcoma - metabolism Rhabdomyosarcoma - pathology Rhabdomyosarcoma, Embryonal - metabolism Rhabdomyosarcoma, Embryonal - pathology Wilms Tumor - metabolism Wilms Tumor - pathology
Myo/Nog cells are identified by their expression of the skeletal muscle specific transcription factor MyoD and the bone morphogenetic protein inhibitor noggin, and binding of the G8 monoclonal antibody. Their release of noggin is critical for morphogenesis and skeletal myogenesis. In the adult, Myo/Nog cells are present in normal tissues, wounds and skin tumors. Myo/Nog cells in the lens give rise to myofibroblasts that synthesize skeletal muscle proteins. The purpose of this study was to screen human lens tissue, rhabdomyosarcoma cell lines, and tissue sections from rhabdomyosarcoma, Wilms and tumors lacking features of skeletal muscle for co-localization of antibodies to Myo/Nog cell markers and the lens beaded filament proteins filensin and CP49. Immunofluorescence localization experiments revealed that Myo/Nog cells of the lens bind antibodies to beaded filament proteins. Co-localization of antibodies to G8, noggin, filensin and CP49 was observed in most RC13 and a subpopulation of RD human rhabdomyosarcoma cell lines. Western blotting with beaded filament antibodies revealed bands of similar molecular weights in RC13 and murine lens cells. Human alveolar, embryonal, pleomorphic and spindle cell rhabdomyosarcomas and Wilms tumors contained a subpopulation of cells immunoreactive for G8, noggin, MyoD and beaded filaments. G8 was also co-localized with filensin mRNA. Staining for beaded filament proteins was not detected in G8 positive cells in leiomyosarcomas, squamous and basal cell carcinomas, syringocarciomas and malignant melanomas. Lens beaded filament proteins were thought to be present only in the lens. Myo/Nog-like cells immunoreactive for beaded filaments may be diagnostic of tumors related to the skeletal muscle lineage.

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Collaboration types
Domestic collaboration
Web of Science research areas
Cell Biology
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