Genetic and cellular evidence of vascular inflammation in neurofibromin-deficient mice and humans

Neurofibromatosis type 1 (NF1) results from mutations in the NF1 tumor suppressor gene, which encodes the protein neurofibromin. NF1 patients display diverse clinical manifestations, including vascular disease, which results from neointima formation and vessel occlusion. However, the pathogenesis of...

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Veröffentlicht in:	The Journal of clinical investigation 2010-03, Vol.120 (3), p.859-870
Hauptverfasser:	Lasater, Elisabeth A, Li, Fang, Bessler, Waylan K, Estes, Myka L, Vemula, Sasidhar, Hingtgen, Cynthia M, Dinauer, Mary C, Kapur, Reuben, Conway, Simon J, Ingram, Jr, David A
Format:	Artikel
Sprache:	eng
Schlagworte:	Adolescent Adult Animals Biomedical research Bone marrow Bone Marrow Cells - metabolism Bone Marrow Cells - pathology Care and treatment Carotid arteries Cytokines Endothelial Cells - metabolism Endothelial Cells - pathology Female Genetic aspects Health aspects Hematopoietic Stem Cell Transplantation Hematopoietic stem cells Homeostasis Humans Inflammation Male Mice Mice, Knockout Muscle, Smooth, Vascular - metabolism Muscle, Smooth, Vascular - pathology Mutation Myocytes, Smooth Muscle - metabolism Myocytes, Smooth Muscle - pathology Neurofibromatosis Neurofibromatosis 1 - genetics Neurofibromatosis 1 - metabolism Neurofibromatosis 1 - pathology Neurofibromin 1 Pathogenesis Proteins Stem cells Transplantation Vasculitis - genetics Vasculitis - metabolism Vasculitis - pathology
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