Publication Date:
1999-12-11
Description:
Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder characterized by increased incidence of benign and malignant tumors of neural crest origin. Mutations that activate the protooncogene ras, such as loss of Nf1, cooperate with inactivating mutations at the p53 tumor suppressor gene during malignant transformation. One hundred percent of mice harboring null Nf1 and p53 alleles in cis synergize to develop soft tissue sarcomas between 3 and 7 months of age. These sarcomas exhibit loss of heterozygosity at both gene loci and express phenotypic traits characteristic of neural crest derivatives and human NF1 malignancies.〈br /〉〈br /〉〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3079436/" target="_blank"〉〈img src="https://static.pubmed.gov/portal/portal3rc.fcgi/4089621/img/3977009" border="0"〉〈/a〉 〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3079436/" target="_blank"〉This paper as free author manuscript - peer-reviewed and accepted for publication〈/a〉〈br /〉〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Vogel, K S -- Klesse, L J -- Velasco-Miguel, S -- Meyers, K -- Rushing, E J -- Parada, L F -- NS34296/NS/NINDS NIH HHS/ -- R01 NS034296/NS/NINDS NIH HHS/ -- R01 NS034296-04/NS/NINDS NIH HHS/ -- New York, N.Y. -- Science. 1999 Dec 10;286(5447):2176-9.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Center for Developmental Biology and Department of Pathology, University of Texas Southwestern Medical Center, 6000 Harry Hines Blvd., Dallas, TX 75235-9133, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/10591653" target="_blank"〉PubMed〈/a〉
Keywords:
Animals
;
Biomarkers, Tumor
;
Cell Differentiation
;
Cell Transformation, Neoplastic
;
Crosses, Genetic
;
*Disease Models, Animal
;
Female
;
*Genes, Neurofibromatosis 1
;
*Genes, p53
;
Heterozygote
;
Humans
;
Loss of Heterozygosity
;
Male
;
Mice
;
Mice, Inbred C57BL
;
Mutation
;
Neural Crest/metabolism/pathology
;
Neurofibromatosis 1/*genetics/*pathology
;
Sarcoma/genetics/*pathology
;
Schwann Cells/metabolism/pathology
;
Tumor Cells, Cultured
Print ISSN:
0036-8075
Electronic ISSN:
1095-9203
Topics:
Biology
,
Chemistry and Pharmacology
,
Computer Science
,
Medicine
,
Natural Sciences in General
,
Physics
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