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Intermediate repeat expansions of TBPand STUB1: Genetic modifier or pure digenic inheritance in spinocerebellar ataxias?

Authors :
Barbier, Mathieu
Davoine, Claire-Sophie
Petit, Emilien
Porché, Maximilien
Guillot-Noel, Léna
Sayah, Sabrina
Fauret, Anne-Laure
Neau, Jean-Philippe
Guyant-Maréchal, Lucie
Deffond, Didier
Tranchant, Christine
Goizet, Cyril
Coarelli, Giulia
Castrioto, Anna
Klebe, Stephan
Ewenczyk, Claire
Heinzmann, Anna
Charles, Perrine
Tchikviladzé, Maya
Van Broeckhoven, Christine
Brice, Alexis
Durr, Alexandra
Source :
Genetics in Medicine; February 2023, Vol. 25 Issue: 2
Publication Year :
2023

Abstract

CAG/CAA repeat expansions in TBP>49are responsible for spinocerebellar ataxia (SCA) type 17 (SCA17). We previously detected cosegregation of STUB1variants causing SCA48 with intermediate alleles of TBPin 2 families. This cosegregation questions the existence of SCA48 as a monogenic disease.

Details

Language :
English
ISSN :
10983600 and 15300366
Volume :
25
Issue :
2
Database :
Supplemental Index
Journal :
Genetics in Medicine
Publication Type :
Periodical
Accession number :
ejs61528280
Full Text :
https://doi.org/10.1016/j.gim.2022.10.009