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Zebrafish Model for NF1

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Abstract

Technological innovations continue to provide investigators with an ever-expanding toolkit for the manipulation of biological systems and the assessment of physiological state. Central to these advances has been the emergence of animal models that accurately recapitulate human disease. These tools have proven invaluable in the transformation of medical investigation from descriptive efforts focusing on natural history and pathology to mechanistic understandings of the consequences that arise from the molecular lesions underpinning these conditions. To this end,Drosophila and murine models of type I neurofibromatosis (NF1) have provided enormous insight into disease pathogenesis and NF1 biology. Despite these successes, presently available animal models of NF1 preclude the ability to efficiently approach a number of fundamental questions facing the NF1 research community. Here, we describe efforts that have culminated in the development of a novel animal model for NF1 that exploits unique features of the zebrafish system and outline new findings regarding NF1 that have resulted from this approach. The zebrafish model offers the ability to perform large-scale chemical and genetic screens in a vertebrate system. This model system will serve as a powerful platform to gain further mechanistic insight into NF1-associated pathology and for the development and testing of new therapies.

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Authors and Affiliations

  1. Department of Cell and Developmental Biology, Penn Cardiovascular Institute, Philadelphia, USA

    Arun Padmanabhan & Jonathan A. Epstein

  2. Institute for Regenerative Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, PA, 19104, USA

    Arun Padmanabhan & Jonathan A. Epstein

  3. 1154 BRB II/III, 421 Curie Blvd, Philadelphia, PA, 19104, USA

    Jonathan A. Epstein

Authors
  1. Arun Padmanabhan

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  2. Jonathan A. Epstein

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Correspondence toJonathan A. Epstein.

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Editors and Affiliations

  1. School of Medicine, Institute of Medical Genetics, Cardiff University, Heath Park Campus, Cardiff, 144XN, United Kingdom

    Meena Upadhyaya

  2. School of Medicine, Institute of Medical Genetics, Cardiff University, Heath Park, Cardiff, CF14 4XN, United Kingdom

    David N. Cooper

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Padmanabhan, A., Epstein, J.A. (2012). Zebrafish Model for NF1. In: Upadhyaya, M., Cooper, D. (eds) Neurofibromatosis Type 1. Springer, Berlin, Heidelberg. https://doi.org/10.1007/978-3-642-32864-0_35

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