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Neurofibromin as a regulator of melanocyte development and differentiation.

Neurofibromin as a regulator of melanocyte development and differentiation. Research Abstract Details 

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  • Neurofibromin as a regulator of melanocyte development and differentiation. Abstract Text:

    ganesh diwakarGanesh Diwakar,deming zhangDeming Zhang,shunlin jiangShunlin Jiang,thomas j hornyakThomas J Hornyak,

    Patients with the genetic disease type I neurofibromatosis (NF1) exhibit characteristic pigmentary lesions associated with loss of a single allele of NF1, encoding the 260 kDa protein neurofibromin. To understand the basis for these pigmentary problems, the properties of melanocytes haploinsufficient for the murine gene Nf1 were studied using Nf1(+/-) knockout mice. We demonstrate that neurofibromin regulates the Kit-Mitf signaling axis in vivo during melanocyte development. Primary Nf1(+/-) melanocytes were purified by FACS to measure melanogenic gene expression. We found that Nf1(+/-) melanocytes exhibit higher levels of melanogenic gene expression than their wild-type counterparts. Both prior to and following Kit stimulation, Nf1(+/-) melanocytes also exhibit increased activation of the MAP kinase pathway compared with primary cells. The melanogenic response of primary melanocytes to Mek inhibition is consistent with the changes observed with Nf1 haploinsufficiency; however, these changes differ from those observed with their immortalized counterparts. The observation that reduction of neurofibromin, either from haploinsufficiency in the case of primary melanocytes or from neurofibromin knockdown in the case of melan-a cells, enhances melanogenic gene expression suggests that neurofibromin plays a dominant role to MEK activity in controlling melanogenic gene expression in murine melanocytes.

    Neurofibromin as a regulator of melanocyte development and differentiation. Publishing Authors By Initials

    g diwakarG Diwakar,d zhangD Zhang,s jiangS Jiang,tj hornyakTJ Hornyak,

    For similar abstracts research abstracts see: abstracts research

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    Neurofibromin as a regulator of melanocyte development and differentiation. Journal Published:

    PUBLICATION TYPE: Journal Article

    Journal: Journal of cell science

    VOLUME: 121

    Page Numbers: 167-77

    Journal Abbreviation: J. Cell. Sci.

    ISSN: 0021-9533

    DAY: 18

    MONTH: 12

    YEAR: 2007

    Neurofibromin as a regulator of melanocyte development and differentiation. Information

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    LANGUAGE: eng

    NlmUniqueID: 52457

    Neurofibromin as a regulator of melanocyte development and differentiation. Keywords Mesh Terms:

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    Grant and Affiliation Information for Neurofibromin as a regulator of melanocyte development and differentiation.

    AFFILIATION: Dermatology Branch, Center for Cancer Research, National Cancer Institute, National Institutes of Health, 10 Center Drive, Bethesda, MD 20892, USA.

    Country: England

    England Research PublicationEngland Research Publication

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    MEDLINETA: J Cell Sci

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