Isolation and characterization of Schwann cells from neurofibromatosis type 2 patients

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Isolation and characterization of Schwann cells from neurofibromatosis type 2 patients. / Rosenbaum, C; Kluwe, L; Mautner, V F; Friedrich, R E; Müller, H W; Hanemann, C O.

In: NEUROBIOL DIS, Vol. 5, No. 1, 01.07.1998, p. 55-64.

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@article{d71f8e875510426f8945067b2a3cdb39,
title = "Isolation and characterization of Schwann cells from neurofibromatosis type 2 patients",
abstract = "Neurofibromatosis type 2 (NF2) is an autosomal dominant disease of the nervous system characterized by multiple schwannomas. The NF2 gene product, termed schwannomin or merlin, was hypothesized to function as a cytoskeleton-membrane linking protein due to homology to members of the protein 4.1 superfamily and to function as a tumor suppressor. We isolated and characterized pure Schwann cell cultures from schwannomas derived from neurofibromatosis 2 patients with identified germline mutations and loss of heterozygosity. We describe striking differences between NF2 and control Schwann cells in morphology, cell-cell contacts, and growth. NF2 Schwann cells form multiple long processes with filopodial and lamellopodial extensions. NF2 Schwann cells lack contact inhibition, grow in multiple layers, and show a higher proliferation rate than control cells. For the first time Schwann cells derived from patients with the NF2 genotype were cultured and characterized in vitro. These cultures are highly valuable for investigating the effects of NF2 mutations and the development of therapies.",
keywords = "Cell Division, Cell Separation, Germ-Line Mutation, Humans, Neurilemmoma, Neurofibromatosis 1, Schwann Cells, Tumor Cells, Cultured",
author = "C Rosenbaum and L Kluwe and Mautner, {V F} and Friedrich, {R E} and M{\"u}ller, {H W} and Hanemann, {C O}",
year = "1998",
month = jul,
day = "1",
doi = "10.1006/nbdi.1998.0179",
language = "English",
volume = "5",
pages = "55--64",
journal = "NEUROBIOL DIS",
issn = "0969-9961",
publisher = "Academic Press Inc.",
number = "1",

}

RIS

TY - JOUR

T1 - Isolation and characterization of Schwann cells from neurofibromatosis type 2 patients

AU - Rosenbaum, C

AU - Kluwe, L

AU - Mautner, V F

AU - Friedrich, R E

AU - Müller, H W

AU - Hanemann, C O

PY - 1998/7/1

Y1 - 1998/7/1

N2 - Neurofibromatosis type 2 (NF2) is an autosomal dominant disease of the nervous system characterized by multiple schwannomas. The NF2 gene product, termed schwannomin or merlin, was hypothesized to function as a cytoskeleton-membrane linking protein due to homology to members of the protein 4.1 superfamily and to function as a tumor suppressor. We isolated and characterized pure Schwann cell cultures from schwannomas derived from neurofibromatosis 2 patients with identified germline mutations and loss of heterozygosity. We describe striking differences between NF2 and control Schwann cells in morphology, cell-cell contacts, and growth. NF2 Schwann cells form multiple long processes with filopodial and lamellopodial extensions. NF2 Schwann cells lack contact inhibition, grow in multiple layers, and show a higher proliferation rate than control cells. For the first time Schwann cells derived from patients with the NF2 genotype were cultured and characterized in vitro. These cultures are highly valuable for investigating the effects of NF2 mutations and the development of therapies.

AB - Neurofibromatosis type 2 (NF2) is an autosomal dominant disease of the nervous system characterized by multiple schwannomas. The NF2 gene product, termed schwannomin or merlin, was hypothesized to function as a cytoskeleton-membrane linking protein due to homology to members of the protein 4.1 superfamily and to function as a tumor suppressor. We isolated and characterized pure Schwann cell cultures from schwannomas derived from neurofibromatosis 2 patients with identified germline mutations and loss of heterozygosity. We describe striking differences between NF2 and control Schwann cells in morphology, cell-cell contacts, and growth. NF2 Schwann cells form multiple long processes with filopodial and lamellopodial extensions. NF2 Schwann cells lack contact inhibition, grow in multiple layers, and show a higher proliferation rate than control cells. For the first time Schwann cells derived from patients with the NF2 genotype were cultured and characterized in vitro. These cultures are highly valuable for investigating the effects of NF2 mutations and the development of therapies.

KW - Cell Division

KW - Cell Separation

KW - Germ-Line Mutation

KW - Humans

KW - Neurilemmoma

KW - Neurofibromatosis 1

KW - Schwann Cells

KW - Tumor Cells, Cultured

U2 - 10.1006/nbdi.1998.0179

DO - 10.1006/nbdi.1998.0179

M3 - SCORING: Journal article

C2 - 9702788

VL - 5

SP - 55

EP - 64

JO - NEUROBIOL DIS

JF - NEUROBIOL DIS

SN - 0969-9961

IS - 1

ER -