Allelic Loss of the NF1 Gene in NF1-Associated Plexiform Neurofibromas
โ Scribed by Lan Kluwe; Reinhard E Friedrich; Victor F Mautner
- Book ID
- 114136912
- Publisher
- Elsevier Science
- Year
- 1999
- Tongue
- English
- Weight
- 153 KB
- Volume
- 113
- Category
- Article
- ISSN
- 0165-4608
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๐ SIMILAR VOLUMES
Neurofibromatosis type 1 (NF1), a common autosomal dominant neurogenetic disorder affecting 1 in 4000 individuals worldwide, results from functional inactivation of the 17q11.2-located NF1 gene. Plexiform neurofibroma (PNF) is a congenital benign tumour present in 30-50% of NF1 patients, which in ab
Neurofibromatosis 1 (NF1) is an autosomal dominant disorder caused by genetic alterations of the NF1 gene on 17q11.2. About 30% of NF1 patients develop plexiform neurofibromas (PNFs), which often cause severe clinical deficits. To determine whether there is a certain genotype underlying PNFs or subt
Neurofibromas, the hallmark of neurofibromatosis 1, are composed mainly of Schwann cells and fibroblasts. Inactivation of both NF1 alleles is the cause of these benign tumors, but it is unknown which cell type is the progenitor. In this study, we selectively cultured Schwann cells from an NF1-associ