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Neonatal gene therapy achieves sustained disease rescue of maple syrup urine disease in mice

Authors :
Clément Pontoizeau
Marcelo Simon-Sola
Clovis Gaborit
Vincent Nguyen
Irina Rotaru
Nolan Tual
Pasqualina Colella
Muriel Girard
Maria-Grazia Biferi
Jean-Baptiste Arnoux
Agnès Rötig
Chris Ottolenghi
Pascale de Lonlay
Federico Mingozzi
Marina Cavazzana
Manuel Schiff
Source :
Nature Communications, Vol 13, Iss 1, Pp 1-13 (2022)
Publication Year :
2022
Publisher :
Nature Portfolio, 2022.

Abstract

Maple syrup urine disease (MSUD) is a rare inborn error of metabolism, which is currently treated with life-long low-protein diet that can be challenging to maintain. Here the authors develop an AAV8-directed gene therapy providing sustainable disease rescue in a mouse model of MSUD.

Subjects

Subjects :
Science

Details

Language :
English
ISSN :
20411723
Volume :
13
Issue :
1
Database :
Directory of Open Access Journals
Journal :
Nature Communications
Publication Type :
Academic Journal
Accession number :
edsdoj.bccd1d42d05b472e924ee795b21fc179
Document Type :
article
Full Text :
https://doi.org/10.1038/s41467-022-30880-w