A novel TSC1 variant associated with tuberous sclerosis and sacrococcygeal teratoma.


Journal

Human genome variation
ISSN: 2054-345X
Titre abrégé: Hum Genome Var
Pays: England
ID NLM: 101652445

Informations de publication

Date de publication:
19 Nov 2020
Historique:
received: 11 07 2020
accepted: 05 10 2020
revised: 15 09 2020
entrez: 10 12 2020
pubmed: 11 12 2020
medline: 11 12 2020
Statut: epublish

Résumé

Tuberous sclerosis complex (TSC) is an autosomal dominant disease associated with tumors and malformed tissues in the brain and other vital organs. We report a novel de novo frameshift variant of the TSC1 gene (c.434dup;p. Ser146Valfs*8) in a child with TSC who initially presented with a sacral teratoma. This previously unreported association between TSC and teratoma has broad implications for the pathophysiology of embryonic tumors and mechanisms underlying cellular differentiation.

Identifiants

pubmed: 33298910
doi: 10.1038/s41439-020-00124-8
pii: 10.1038/s41439-020-00124-8
pmc: PMC7677537
doi:

Types de publication

Journal Article

Langues

eng

Pagination

39

Subventions

Organisme : U.S. Department of Defense (United States Department of Defense)
ID : W81XWH-09-1-0088

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Auteurs

Saba Ahmad (S)

Department of Pediatrics, The Division of Pediatric Neurology, University of Illinois at Chicago, Chicago, IL, USA.

Luis Manon (L)

Department of Pathology, University of Illinois at Chicago, Chicago, IL, USA.

Gifty Bhat (G)

Department of Pediatrics, The Division of Genetics, University of Illinois at Chicago, Chicago, IL, USA.

Jerry Machado (J)

Prevention Genetics, Marshfield, WI, 54449, USA.

Alice Zalan (A)

Department of Pediatrics, The Division of Genetics, University of Illinois at Chicago, Chicago, IL, USA.

Nikolas Mata-Machado (N)

Department of Pediatrics, The Division of Pediatric Neurology, University of Illinois at Chicago, Chicago, IL, USA.

Steven Garzon (S)

Department of Pathology, University of Illinois at Chicago, Chicago, IL, USA.

Akira Yoshii (A)

Department of Pediatrics, The Division of Pediatric Neurology, University of Illinois at Chicago, Chicago, IL, USA. ayoshii@uic.edu.
Department of Anatomy and Cell Biology, University of Illinois at Chicago, Chicago, IL, USA. ayoshii@uic.edu.

Classifications MeSH