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Mitochondrial encephalomyopathy and retinoblastoma explained by compound heterozygosity of SUCLA2 point mutation and 13q14 deletion

Mutations in SUCLA2, encoding the ß-subunit of succinyl-CoA synthetase of Krebs cycle, are one cause of mitochondrial DNA depletion syndrome. Patients have been reported to have severe progressive childhood-onset encephalomyopathy, and methylmalonic aciduria, often leading to death in childhood. We...

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Détails bibliographiques
Publié dans:Eur J Hum Genet
Auteurs principaux: Matilainen, Sanna, Isohanni, Pirjo, Euro, Liliya, Lönnqvist, Tuula, Pihko, Helena, Kivelä, Tero, Knuutila, Sakari, Suomalainen, Anu
Format: Artigo
Langue:Inglês
Publié: Nature Publishing Group 2015
Sujets:
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC4326715/
https://ncbi.nlm.nih.gov/pubmed/24986829
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/ejhg.2014.128
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