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- W2083395199 abstract "Neurofibromatosis type 1 (NF1), the most common genetic disorder affecting the human nervous system, is characterized by the development of multiple benign Schwann cell tumors in skin and large peripheral nerves. These neoplasms, which are termed dermal and plexiform neurofibromas respectively, have distinct clinical courses; of particular note, plexiform, but not dermal, neurofibromas often undergo malignant progression to form malignant peripheral nerve sheath tumors (MPNSTs), the most common malignancy occurring in NF1 patients. In recent years, a number of genetically engineered mouse models have been created to investigate the molecular mechanisms driving the pathogenesis of these tumors. These models have been designed to address key questions including: (1) whether NF1 loss in the Schwann cell lineage is essential for tumorigenesis; (2) what cell type(s) in the Schwann cell lineage gives rise to dermal neurofibromas, plexiform neurofibromas and MPNSTs; (3) how the tumor microenvironment contributes to neoplasia; (4) what additional mutations contribute to neurofibroma-MPNST progression; (5) what role different neurofibromin-regulated Ras proteins play in this process and (6) how dysregulated growth factor signaling facilitates PNS tumorigenesis. In this review, we summarize the major findings from each of these models and their limitations as well as how discrepancies between these models may be reconciled. We also discuss how information gleaned from these models can be synthesized to into a comprehensive model of tumor formation in peripheral nervous system and consider several of the major questions that remain unanswered about this process." @default.
- W2083395199 created "2016-06-24" @default.
- W2083395199 creator A5071472484 @default.
- W2083395199 creator A5090060992 @default.
- W2083395199 date "2012-05-01" @default.
- W2083395199 modified "2023-09-25" @default.
- W2083395199 title "Genetically engineered mouse models shed new light on the pathogenesis of neurofibromatosis type I-related neoplasms of the peripheral nervous system" @default.
- W2083395199 cites W1584418808 @default.
- W2083395199 cites W1585517331 @default.
- W2083395199 cites W1673160816 @default.
- W2083395199 cites W1791732177 @default.
- W2083395199 cites W1812955231 @default.
- W2083395199 cites W1863238068 @default.
- W2083395199 cites W1882985910 @default.
- W2083395199 cites W1967125902 @default.
- W2083395199 cites W1971602793 @default.
- W2083395199 cites W1971656848 @default.
- W2083395199 cites W1974133930 @default.
- W2083395199 cites W1975063093 @default.
- W2083395199 cites W1979154683 @default.
- W2083395199 cites W1983673751 @default.
- W2083395199 cites W1990880437 @default.
- W2083395199 cites W1991276398 @default.
- W2083395199 cites W1992013240 @default.
- W2083395199 cites W1992738872 @default.
- W2083395199 cites W1996539839 @default.
- W2083395199 cites W2000570698 @default.
- W2083395199 cites W2004377723 @default.
- W2083395199 cites W2005322331 @default.
- W2083395199 cites W2007676864 @default.
- W2083395199 cites W2008118174 @default.
- W2083395199 cites W2008471038 @default.
- W2083395199 cites W2009235369 @default.
- W2083395199 cites W2017444651 @default.
- W2083395199 cites W2021304700 @default.
- W2083395199 cites W2022237108 @default.
- W2083395199 cites W2022971724 @default.
- W2083395199 cites W2023947962 @default.
- W2083395199 cites W2024616525 @default.
- W2083395199 cites W2025561101 @default.
- W2083395199 cites W2025722101 @default.
- W2083395199 cites W202730752 @default.
- W2083395199 cites W2027339273 @default.
- W2083395199 cites W2028757936 @default.
- W2083395199 cites W2032969727 @default.
- W2083395199 cites W2034303286 @default.
- W2083395199 cites W2035961437 @default.
- W2083395199 cites W2039752032 @default.
- W2083395199 cites W2040142253 @default.
- W2083395199 cites W2042211545 @default.
- W2083395199 cites W2042346521 @default.
- W2083395199 cites W2042552060 @default.
- W2083395199 cites W2044210875 @default.
- W2083395199 cites W2047818921 @default.
- W2083395199 cites W2047826668 @default.
- W2083395199 cites W2054674800 @default.
- W2083395199 cites W2055729102 @default.
- W2083395199 cites W2057955140 @default.
- W2083395199 cites W2060632243 @default.
- W2083395199 cites W2060696651 @default.
- W2083395199 cites W2068427129 @default.
- W2083395199 cites W2068718620 @default.
- W2083395199 cites W2069886811 @default.
- W2083395199 cites W2071743372 @default.
- W2083395199 cites W2071893342 @default.
- W2083395199 cites W2082057360 @default.
- W2083395199 cites W2082277907 @default.
- W2083395199 cites W2082888699 @default.
- W2083395199 cites W2087089518 @default.
- W2083395199 cites W2089794529 @default.
- W2083395199 cites W2091514845 @default.
- W2083395199 cites W2094200242 @default.
- W2083395199 cites W2094330297 @default.
- W2083395199 cites W2095237073 @default.
- W2083395199 cites W2105849179 @default.
- W2083395199 cites W2121733985 @default.
- W2083395199 cites W2121913090 @default.
- W2083395199 cites W2128858154 @default.
- W2083395199 cites W2130521145 @default.
- W2083395199 cites W2135833883 @default.
- W2083395199 cites W2138616826 @default.
- W2083395199 cites W2142662937 @default.
- W2083395199 cites W2143651667 @default.
- W2083395199 cites W2145481360 @default.
- W2083395199 cites W2146579564 @default.
- W2083395199 cites W2146978754 @default.
- W2083395199 cites W2154151857 @default.
- W2083395199 cites W2159096148 @default.
- W2083395199 cites W2159562139 @default.
- W2083395199 cites W2161024497 @default.
- W2083395199 cites W2167149037 @default.
- W2083395199 cites W228407023 @default.
- W2083395199 cites W2330881865 @default.
- W2083395199 cites W2339412923 @default.
- W2083395199 cites W2410571468 @default.
- W2083395199 cites W4239588487 @default.
- W2083395199 cites W4243003607 @default.
- W2083395199 cites W4296759778 @default.