Journal
CANCER CELL
Volume 13, Issue 2, Pages 129-140Publisher
CELL PRESS
DOI: 10.1016/j.ccr.2008.01.003
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Funding
- NHLBI NIH HHS [T32 HL079995, R01 HL077342-04, R01 HL077342] Funding Source: Medline
- NINDS NIH HHS [R01 NS040750, R01 NS40750, R01 NS040750-06, F30 NS049761, R01 NS040750-08, R01 NS040750-07, R01 NS040750-01] Funding Source: Medline
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Neurofibromatosis is caused by the loss of neurofibromin (Nf1), leading to peripheral nervous system (PNS) tumors, including neurofibromas and malignant peripheral nerve sheath tumors (MPNSTs). A long-standing question has been whether these tumors arise from neural crest stem cells (NCSCs) or differentiated glia. Germline or conditional Nf1 deficiency caused a transient increase in NCSC frequency and self-renewal in most regions of the fetal PNS. However, Nf1-deficient NCSCs did not persist postnatally in regions of the PNS that developed tumors and could not form tumors upon transplantation into adult nerves. Adult P0a-Cre(+)Nf1(fl/-) mice developed neurofibromas, and Nf1(+/-)Ink4a/Arf(-/-) and Nf1/p53(+/-) mice developed MPNSTs, but NCSCs did not persist postnatally in affected locations in these mice. Tumors appeared to arise from differentiated glia, not NCSCs.
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