title: The generation and characterization of a cell line derived from a sporadic renal angiomyolipoma: use of telomerase to obtain stable populations of cells from benign neoplasms.
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.
authors.affiliation: Department of Dermatology, Emory University School of Medicine, 1639 Pierce Drive, Atlanta, GA 30322, USA. jarbise@emory.edu
authors.forename: J L
authors.initials: JL
authors.lastname: Arbiser
authors.forename: R
authors.initials: R
authors.lastname: Yeung
authors.forename: S W
authors.initials: SW
authors.lastname: Weiss
authors.forename: Z K
authors.initials: ZK
authors.lastname: Arbiser
authors.forename: M B
authors.initials: MB
authors.lastname: Amin
authors.forename: C
authors.initials: C
authors.lastname: Cohen
authors.forename: D
authors.initials: D
authors.lastname: Frank
authors.forename: S
authors.initials: S
authors.lastname: Mahajan
authors.forename: G S
authors.initials: GS
authors.lastname: Herron
authors.forename: J
authors.initials: J
authors.lastname: Yang
authors.forename: H
authors.initials: H
authors.lastname: Onda
authors.forename: H B
authors.initials: HB
authors.lastname: Zhang
authors.forename: X
authors.initials: X
authors.lastname: Bai
authors.forename: E
authors.initials: E
authors.lastname: Uhlmann
authors.forename: A
authors.initials: A
authors.lastname: Loehr
authors.forename: H
authors.initials: H
authors.lastname: Northrup
authors.forename: P
authors.initials: P
authors.lastname: Au
authors.forename: I
authors.initials: I
authors.lastname: Davis
authors.forename: D E
authors.initials: DE
authors.lastname: Fisher
authors.forename: D H
authors.initials: DH
authors.lastname: Gutmann
mesh_terms: D000199:Actins / Q000032:analysis; D000273:Adipose Tissue / Q000166:cytology / Q000473:pathology; D018207:Angiomyolipoma / Q000235:genetics / Q000473:pathology* / Q000648:ultrastructure; D000952:Antigens, Polyomavirus Transforming / Q000235:genetics; D018929:Cell Culture Techniques / Q000379:methods; D006801:Humans; D007150:Immunohistochemistry; D007680:Kidney Neoplasms / Q000235:genetics / Q000473:pathology* / Q000648:ultrastructure; D008544:Melanocytes / Q000166:cytology / Q000473:pathology; D020928:Mitogen-Activated Protein Kinases / Q000032:analysis; D009130:Muscle, Smooth / Q000166:cytology / Q000473:pathology; D010641:Phenotype; D010766:Phosphorylation; D011506:Proteins / Q000032:analysis / Q000235:genetics; D018160:Receptors, Cytoplasmic and Nuclear / Q000032:analysis / Q000378:metabolism; D012097:Repressor Proteins / Q000032:analysis / Q000235:genetics; D013539:Simian virus 40 / Q000235:genetics; D019098:Telomerase / Q000032:analysis / Q000378:metabolism*; D014157:Transcription Factors / Q000032:analysis / Q000378:metabolism; D014162:Transfection; D000077004:Tuberous Sclerosis Complex 1 Protein; D000077005:Tuberous Sclerosis Complex 2 Protein; D014407:Tumor Cells, Cultured; D025521:Tumor Suppressor Proteins
chemical_list: D000199:Actins; D000952:Antigens, Polyomavirus Transforming; D011506:Proteins; D018160:Receptors, Cytoplasmic and Nuclear; D012097:Repressor Proteins; D014157:Transcription Factors; D000077004:Tuberous Sclerosis Complex 1 Protein; D000077005:Tuberous Sclerosis Complex 2 Protein; D025521:Tumor Suppressor Proteins; D020928:Mitogen-Activated Protein Kinases; D019098:Telomerase