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Article

The Risk of Transmission of Genetic Prion Diseases is Greater Than 50.

European journal of neurology - 1 Dec 2025

Kortazar-Zubizarreta Izaro, Manero-Azua Africa, Eraña Hasier, Aguire Urko, Castilla Joaquín, Perez de Nanclares Guiomar

Abstract excerpt

BACKGROUND: Inherited prion diseases follow autosomal dominant inheritance with a theoretical 50% transmission risk per pregnancy. Transmission ratio distortion (TRD)-deviation from expected Mendelian ratios-has been documented in other genetic disorders but never systematically studied in prionopathies. We aimed to determine whether TRD occurs in families with inherited prion diseases-specifically familial...

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Identifiers

PMID: 41351309
DOI: 10.1111/ene.70455
PMCID: PMC12680899

Genes

TRD

Provenance

PubMed metadata and Literature Corpus relationships. Corpus snapshot updated 4 Sept 2026.

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