Insights into the pathogenesis of neurofibromatosis 1 vasculopathy.

作者: SJ Hamilton , JM Friedman

DOI: 10.1034/J.1399-0004.2000.580501.X

关键词: PathogenesisBlood vesselPathophysiologyMyocyteNeurofibromin 1PathologyEndotheliumMedicineNeurofibromatosisVascular disease

摘要: Intrinsic lesions of arterial walls are an important manifestation neurofibromatosis 1 (NF1). Neurofibromin is expressed in blood vessel endothelial and smooth muscle cells, NF1 vasculopathy may result from alteration neurofibromin function these cells. Elucidation the role maintenance repair vessels lead to novel approaches treatment vascular disease general.

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