کد مقاله کد نشریه سال انتشار مقاله انگلیسی نسخه تمام متن
2108056 1083719 2008 12 صفحه PDF دانلود رایگان
عنوان انگلیسی مقاله ISI
Induction of Abnormal Proliferation by Nonmyelinating Schwann Cells Triggers Neurofibroma Formation
موضوعات مرتبط
علوم زیستی و بیوفناوری بیوشیمی، ژنتیک و زیست شناسی مولکولی تحقیقات سرطان
پیش نمایش صفحه اول مقاله
Induction of Abnormal Proliferation by Nonmyelinating Schwann Cells Triggers Neurofibroma Formation
چکیده انگلیسی

SummaryRecent evidence suggests that alterations in the self-renewal program of stem/progenitor cells can cause tumorigenesis. By utilizing genetically engineered mouse models of neurofibromatosis type 1 (NF1), we demonstrated that plexiform neurofibroma, the only benign peripheral nerve sheath tumor with potential for malignant transformation, results from Nf1 deficiency in fetal stem/progenitor cells of peripheral nerves. Surprisingly, this did not cause hyperproliferation or tumorigenesis in early postnatal period. Instead, peripheral nerve development appeared largely normal in the absence of Nf1 except for abnormal Remak bundles, the nonmyelinated axon-Schwann cell unit, identified in postnatal mutant nerves. Subsequent degeneration of abnormal Remak bundles was accompanied by initial expansion of nonmyelinating Schwann cells. We suggest abnormally differentiated Remak bundles as a cell of origin for plexiform neurofibroma.

ناشر
Database: Elsevier - ScienceDirect (ساینس دایرکت)
Journal: - Volume 13, Issue 2, 5 February 2008, Pages 117–128
نویسندگان
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