Abstract
Angiomyolipomas are benign tumors of the kidney derived from putative perivascular epithelioid cells, that may undergo differentiation into cells with features of melanocytes, smooth muscle, and fat. To gain further insight into angiomyolipomas, we have generated the first human angiomyolipoma cell line by sequential introduction of SV40 large T antigen and human telomerase into human angiomyolipoma cells. These cells show phenotypic characteristics of angiomyolipomas, namely differentiation markers of smooth muscle (smooth muscle actin), adipose tissue (peroxisome proliferator-activator receptor gamma, PPARgamma), and melanocytes (microophthalmia, MITF), thus demonstrating that a single cell type can exhibit all of these phenotypes. These cells should serve as a valuable tool to elucidate signal transduction pathways underlying renal angiomyolipomas.
Publication types
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Research Support, U.S. Gov't, P.H.S.
MeSH terms
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Actins / analysis
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Adipose Tissue / cytology
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Adipose Tissue / pathology
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Angiomyolipoma / genetics
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Angiomyolipoma / pathology*
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Angiomyolipoma / ultrastructure
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Antigens, Polyomavirus Transforming / genetics
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Cell Culture Techniques / methods
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Humans
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Immunohistochemistry
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Kidney Neoplasms / genetics
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Kidney Neoplasms / pathology*
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Kidney Neoplasms / ultrastructure
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Melanocytes / cytology
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Melanocytes / pathology
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Mitogen-Activated Protein Kinases / analysis
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Muscle, Smooth / cytology
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Muscle, Smooth / pathology
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Phenotype
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Phosphorylation
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Proteins / analysis
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Proteins / genetics
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Receptors, Cytoplasmic and Nuclear / analysis
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Receptors, Cytoplasmic and Nuclear / metabolism
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Repressor Proteins / analysis
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Repressor Proteins / genetics
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Simian virus 40 / genetics
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Telomerase / analysis
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Telomerase / metabolism*
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Transcription Factors / analysis
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Transcription Factors / metabolism
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Transfection
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Tuberous Sclerosis Complex 1 Protein
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Tuberous Sclerosis Complex 2 Protein
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Tumor Cells, Cultured
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Tumor Suppressor Proteins
Substances
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Actins
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Antigens, Polyomavirus Transforming
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Proteins
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Receptors, Cytoplasmic and Nuclear
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Repressor Proteins
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Transcription Factors
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Tuberous Sclerosis Complex 1 Protein
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Tuberous Sclerosis Complex 2 Protein
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Tumor Suppressor Proteins
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Mitogen-Activated Protein Kinases
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Telomerase