Cellular mechanisms of heterogeneity in NF2-mutant schwannoma.


Journal

Nature communications
ISSN: 2041-1723
Titre abrégé: Nat Commun
Pays: England
ID NLM: 101528555

Informations de publication

Date de publication:
21 03 2023
Historique:
received: 01 10 2021
accepted: 06 03 2023
entrez: 22 3 2023
pubmed: 23 3 2023
medline: 24 3 2023
Statut: epublish

Résumé

Schwannomas are common sporadic tumors and hallmarks of familial neurofibromatosis type 2 (NF2) that develop predominantly on cranial and spinal nerves. Virtually all schwannomas result from inactivation of the NF2 tumor suppressor gene with few, if any, cooperating mutations. Despite their genetic uniformity schwannomas exhibit remarkable clinical and therapeutic heterogeneity, which has impeded successful treatment. How heterogeneity develops in NF2-mutant schwannomas is unknown. We have found that loss of the membrane:cytoskeleton-associated NF2 tumor suppressor, merlin, yields unstable intrinsic polarity and enables Nf2

Identifiants

pubmed: 36944680
doi: 10.1038/s41467-023-37226-0
pii: 10.1038/s41467-023-37226-0
pmc: PMC10030849
doi:

Substances chimiques

Neurofibromin 2 0

Types de publication

Journal Article Research Support, U.S. Gov't, Non-P.H.S. Research Support, N.I.H., Extramural

Langues

eng

Sous-ensembles de citation

IM

Pagination

1559

Subventions

Organisme : NIH HHS
ID : S10 OD025017
Pays : United States

Informations de copyright

© 2023. The Author(s).

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Auteurs

Christine Chiasson-MacKenzie (C)

Massachusetts General Hospital Cancer Center, Harvard Medical School, 149 13th Street, Charlestown, MA, 02129, USA.
Department of Pathology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA, 02114, USA.

Jeremie Vitte (J)

Department of Head and Neck Surgery, David Geffen School of Medicine at UCLA and Jonsson Comprehensive Cancer Center (JCCC), University of California Los Angeles, Los Angeles, CA, 90095, USA.

Ching-Hui Liu (CH)

Massachusetts General Hospital Cancer Center, Harvard Medical School, 149 13th Street, Charlestown, MA, 02129, USA.
Department of Pathology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA, 02114, USA.

Emily A Wright (EA)

Massachusetts General Hospital Cancer Center, Harvard Medical School, 149 13th Street, Charlestown, MA, 02129, USA.
Department of Pathology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA, 02114, USA.

Elizabeth A Flynn (EA)

Massachusetts General Hospital Cancer Center, Harvard Medical School, 149 13th Street, Charlestown, MA, 02129, USA.
Center for Engineering in Medicine and BioMEMS Resource Center, Surgical Services, Massachusetts General Hospital, Harvard Medical School, 114 16th Street, Charlestown, MA, 02129, USA.

Shannon L Stott (SL)

Massachusetts General Hospital Cancer Center, Harvard Medical School, 149 13th Street, Charlestown, MA, 02129, USA.
Center for Engineering in Medicine and BioMEMS Resource Center, Surgical Services, Massachusetts General Hospital, Harvard Medical School, 114 16th Street, Charlestown, MA, 02129, USA.

Marco Giovannini (M)

Department of Head and Neck Surgery, David Geffen School of Medicine at UCLA and Jonsson Comprehensive Cancer Center (JCCC), University of California Los Angeles, Los Angeles, CA, 90095, USA.

Andrea I McClatchey (AI)

Massachusetts General Hospital Cancer Center, Harvard Medical School, 149 13th Street, Charlestown, MA, 02129, USA. mcclatch@helix.mgh.harvard.edu.
Department of Pathology, Massachusetts General Hospital, Harvard Medical School, 55 Fruit Street, Boston, MA, 02114, USA. mcclatch@helix.mgh.harvard.edu.

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