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Genotype-Phenotype Correlation in Primary Carnitine Deficiency

Primary carnitine deficiency is caused by defective OCTN2 carnitine transporters encoded by the SLC22A5 gene. Lack of carnitine impairs fatty acid oxidation resulting in hypoketotic hypoglycemia, hepatic encephalopathy, skeletal and cardiac myopathy. Recently, asymptomatic mothers with primary carni...

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Détails bibliographiques
Auteurs principaux: Rose, Emily Cornforth, di San Filippo, Cristina Amat, Ndukwe Erlingsson, Uzochi C., Ardon, Orly, Pasquali, Marzia, Longo, Nicola
Format: Artigo
Langue:Inglês
Publié: 2011
Sujets:
Accès en ligne:https://ncbi.nlm.nih.gov/pmc/articles/PMC3240685/
https://ncbi.nlm.nih.gov/pubmed/21922592
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/humu.21607
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