Genes Chromosomes Cancer. 1999 Mar;24(3):283-5.

Loss of NF1 allele in Schwann cells but not in fibroblasts derived from an NF1-associated neurofibroma.

Source

Department of Neurosurgery, University Hospital Eppendorf, Hamburg, Germany. kluwe@uke.uni-hamburg.de

Abstract

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-associated neurofibroma. Fibroblasts were also obtained by culturing the tumor cells under standard conditions. Using four intragenic markers, we genotyped the NF1 locus in the original tumor and in the derived Schwann cells and fibroblasts. Loss of heterozygosity for two informative markers, which indicates loss of one NF1 allele, was found in Schwann cells but not in fibroblasts. This result suggests that genetic alterations of the NF1 gene in Schwann cells are responsible for the development of neurofibromas.

PMID:: 10451710; [PubMed - indexed for MEDLINE]

Publication Types, MeSH Terms

Publication Types

Research Support, Non-U.S. Gov't

MeSH Terms

LinkOut - more resources

Full Text Sources

Other Literature Sources

Labome Researcher Resource - ExactAntigen/Labome

Medical

Supplemental Content

Save items

Related citations in PubMed

Single-cell PCR performed with neurofibroma Schwann cells reveals the presence of both alleles of the neurofibromatosis type 1 (NF1) gene. [Hum Genet. 1995]
Single-cell PCR performed with neurofibroma Schwann cells reveals the presence of both alleles of the neurofibromatosis type 1 (NF1) gene.
Stark M, Assum G, Krone W. Hum Genet. 1995 Nov; 96(5):619-23.
Schwann cells harbor the somatic NF1 mutation in neurofibromas: evidence of two different Schwann cell subpopulations. [Hum Mol Genet. 2000]
Schwann cells harbor the somatic NF1 mutation in neurofibromas: evidence of two different Schwann cell subpopulations.
Serra E, Rosenbaum T, Winner U, Aledo R, Ars E, Estivill X, Lenard HG, Lázaro C. Hum Mol Genet. 2000 Dec 12; 9(20):3055-64.
Neurofibromas in NF1: Schwann cell origin and role of tumor environment. [Science. 2002]
Neurofibromas in NF1: Schwann cell origin and role of tumor environment.
Zhu Y, Ghosh P, Charnay P, Burns DK, Parada LF. Science. 2002 May 3; 296(5569):920-2.
Review Molecular, genetic, and cellular pathogenesis of neurofibromas and surgical implications. [Neurosurgery. 2006]
Review Molecular, genetic, and cellular pathogenesis of neurofibromas and surgical implications.
Gottfried ON, Viskochil DH, Fults DW, Couldwell WT. Neurosurgery. 2006 Jan; 58(1):1-16; discussion 1-16.
Review Thinking beyond the tumor cell: Nf1 haploinsufficiency in the tumor environment. [Cancer Cell. 2002]
Review Thinking beyond the tumor cell: Nf1 haploinsufficiency in the tumor environment.
McLaughlin ME, Jacks T. Cancer Cell. 2002 Jun; 1(5):408-10.

See reviews... See all...

Cited by 16 PubMed Central articles

Dissecting loss of heterozygosity (LOH) in neurofibromatosis type 1-associated neurofibromas: Importance of copy neutral LOH. [Hum Mutat. 2011]
Dissecting loss of heterozygosity (LOH) in neurofibromatosis type 1-associated neurofibromas: Importance of copy neutral LOH.
Garcia-Linares C, Fernández-Rodríguez J, Terribas E, Mercadé J, Pros E, Benito L, Benavente Y, Capellà G, Ravella A, Blanco I, et al. Hum Mutat. 2011 Jan; 32(1):78-90.
A patient with neurofibromatosis type 1 and Charcot-Marie-Tooth disease type 1B. [Muscle Nerve. 2010]
A patient with neurofibromatosis type 1 and Charcot-Marie-Tooth disease type 1B.
Lancaster E, Elman LB, Scherer SS. Muscle Nerve. 2010 Apr; 41(4):555-8.
Differential MSH2 promoter methylation in blood cells of Neurofibromatosis type 1 (NF1) patients. [Eur J Hum Genet. 2010]
Differential MSH2 promoter methylation in blood cells of Neurofibromatosis type 1 (NF1) patients.
Titze S, Peters H, Währisch S, Harder T, Guse K, Buske A, Tinschert S, Harder A. Eur J Hum Genet. 2010 Jan; 18(1):81-7.

See all...

Related information

Related Citations
Calculated set of PubMed citations closely related to the selected article(s) retrieved using a word weight algorithm. Related articles are displayed in ranked order from most to least relevant, with the “linked from” citation displayed first.
Cited in PMC
Full-text articles in the PubMed Central Database that cite the current articles.

Recent activity

Clear Turn Off Turn On

Loss of NF1 allele in Schwann cells but not in fibroblasts derived from an NF1-a...
Loss of NF1 allele in Schwann cells but not in fibroblasts derived from an NF1-associated neurofibroma.
Genes Chromosomes Cancer. 1999 Mar ;24(3):283-5.

PubMed
Do HPV-negative cervical carcinomas exist?--revisited.
Do HPV-negative cervical carcinomas exist?--revisited.
J Pathol. 1999 Sep ;189(1):1-3.

PubMed
The prevalence of hepatitis C virus infection in the United States, 1988 through...
The prevalence of hepatitis C virus infection in the United States, 1988 through 1994.
N Engl J Med. 1999 Aug 19 ;341(8):556-62.

PubMed
Cigarette smoking.
Cigarette smoking.
J Natl Cancer Inst. 1999 Aug 18 ;91(16):1365-75.

PubMed
Registration of the rod is not critical for the phosphorylation-dependent regula...
Registration of the rod is not critical for the phosphorylation-dependent regulation of smooth muscle myosin.
Biochemistry. 1999 Aug 17 ;38(33):10768-74.

PubMed

Your browsing activity is empty.

Activity recording is turned off.

Turn recording back on

PubMed

Result Filters

Display Settings:

Send to: