title: The growth regulation of neurofibroma cells in neurofibromatosis type-1: increased responses to PDGF-BB and TGF-beta 1.
abstract: In neurofibromatosis type-1 (NF-1), abnormal growth regulation may be related to the formation of multiple neurofibromas. We investigated the growth responses of neurofibroma-derived cells (NF cells) and control skin fibroblasts to various growth factors. The responses to platelet-derived growth factor (PDGF-BB) and transforming growth factor-beta 1 (TGF-beta 1) in NF cells were significantly greater than those in control fibroblasts. The increased response to PDGF-BB in NF cells was accompanied by an increased number of PDGF beta receptors, which was demonstrated by both 125I PDGF-BB binding assay and immunoblotting analysis. The increased response to TGF-beta 1 was assumed to be mediated through PDGF-like protein induction; TGF-beta 1-treated NF cells produced greater amounts of 36-kD PDGF-like protein than TGF-beta 1-treated control fibroblasts. These observations suggest that certain growth factors, e.g., PDGF-BB and TGF-beta, may play some role in the development of neurofibromas in NF-1.
authors.affiliation: Department of Dermatology, Faculty of Medicine, University of Tokyo, Japan.
authors.forename: T
authors.initials: T
authors.lastname: Kadono
authors.forename: Y
authors.initials: Y
authors.lastname: Soma
authors.forename: K
authors.initials: K
authors.lastname: Takehara
authors.forename: H
authors.initials: H
authors.lastname: Nakagawa
authors.forename: Y
authors.initials: Y
authors.lastname: Ishibashi
authors.forename: K
authors.initials: K
authors.lastname: Kikuchi
mesh_terms: D000077214:Becaplermin; D002455:Cell Division / Q000187:drug effects*; D002478:Cells, Cultured; D005347:Fibroblasts / Q000166:cytology / Q000187:drug effects / Q000473:pathology; D006801:Humans; D015151:Immunoblotting; D007700:Kinetics; D009455:Neurofibroma / Q000473:pathology*; D009456:Neurofibromatosis 1 / Q000473:pathology*; D010982:Platelet-Derived Growth Factor / Q000378:metabolism / Q000494:pharmacology*; D020574:Proto-Oncogene Proteins c-sis; D017479:Receptors, Platelet-Derived Growth Factor / Q000302:isolation & purification / Q000378:metabolism; D011994:Recombinant Proteins / Q000378:metabolism / Q000494:pharmacology; D012867:Skin / Q000166:cytology* / Q000187:drug effects / Q000473:pathology; D012878:Skin Neoplasms / Q000473:pathology*; D013936:Thymidine / Q000378:metabolism; D016212:Transforming Growth Factor beta / Q000494:pharmacology*; D014407:Tumor Cells, Cultured
chemical_list: D010982:Platelet-Derived Growth Factor; D020574:Proto-Oncogene Proteins c-sis; D011994:Recombinant Proteins; D016212:Transforming Growth Factor beta; D000077214:Becaplermin; D017479:Receptors, Platelet-Derived Growth Factor; D013936:Thymidine