Recent developments in neurofibromatoses and RASopathies: management, diagnosis and current and future therapeutic avenues

Am J Med Genet A. 2015 Jan;167A(1):1-10. doi: 10.1002/ajmg.a.36793. Epub 2014 Nov 12.

Abstract

Neurofibromatosis type 1 (NF1) was the first RASopathy and is now one of many RASopathies that are caused by germline mutations in genes that encode components of the Ras/mitogen-activated protein kinase (MAPK) pathway. Their common underlying pathogenetic etiology causes significant overlap in phenotypic features which includes craniofacial dysmorphology, cardiac, cutaneous, musculoskeletal, GI and ocular abnormalities, and a predisposition to cancer. The proceedings from the symposium "Recent Developments in Neurofibromatoses (NF) and RASopathies: Management, Diagnosis and Current and Future Therapeutic Avenues" chronicle this timely and topical clinical translational research symposium. The overarching goal was to bring together clinicians, basic scientists, physician-scientists, advocate leaders, trainees, students and individuals with Ras pathway syndromes to discuss the most state-of-the-art basic science and clinical issues in an effort to spark collaborations directed towards the best practices and therapies for individuals with RASopathies.

Keywords: Costello syndrome; Legius syndrome; Noonan syndrome; RASopathy; Ras/MAPK; capillary malformation-AV malformation syndrome; cardio-facio-cutaneous syndrome; neurofibromatosis; signal transduction pathway; therapy.

Publication types

  • Congress
  • Research Support, N.I.H., Extramural
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Clinical Trials as Topic
  • Disease Models, Animal
  • Humans
  • Magnetic Resonance Imaging
  • Mice
  • Mutation / genetics
  • Neurofibromatoses / diagnosis*
  • Neurofibromatoses / therapy*
  • Syndrome
  • Tumor Burden
  • ras Proteins / genetics*

Substances

  • ras Proteins