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Functional and Molecular Studies in Primary Carnitine Deficiency

Primary carnitine deficiency is caused by a defect in the OCTN2 carnitine transporter encoded by the SLC22A5 gene. It can cause hypoketotic hypoglycemia or cardiomyopathy in children, and sudden death in children and adults. Fibroblasts from affected patients have reduced carnitine transport. We eva...

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Detalles Bibliográficos
Publicado en:Hum Mutat
Main Authors: Frigeni, Marta, Balakrishnan, Bijina, Yin, Xue, Calderon, Fernanda R.O., Mao, Rong, Pasquali, Marzia, Longo, Nicola
Formato: Artigo
Idioma:Inglês
Publicado: 2017
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Acceso en liña:https://ncbi.nlm.nih.gov/pmc/articles/PMC5665702/
https://ncbi.nlm.nih.gov/pubmed/28841266
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/humu.23315
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