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Technical Advances |













From the Departments of Dermatology
* and Pathology,
Emory University School of Medicine and the Winship Cancer Institute, Atlanta, Georgia; the Department of Internal Medicine,
University of Washington School of Medicine, Seattle, Washington; the Departments of Adult Oncology
and Pediatric
Oncology,

Dana Farber Cancer Institute and the Harvard Medical School, Boston, Massachusetts; the Department of Dermatology,
¶ Stanford University School of Medicine, Stanford, California; the Department of Neurology,
|| Washington University School of Medicine, St.
Louis, Missouri; and the Department of Pediatrics/Medical
Genetics,
** University of TexasHouston Division of Pediatric Hematology/Oncology, Houston, Texas
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
, PPAR
), 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.
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