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Very long-/ and long Chain-3-Hydroxy Acyl CoA Dehydrogenase Deficiency correlates with deregulation of the mitochondrial fusion/fission machinery

Children diagnosed with Long-Chain-3-Hydroxy-Acyl-CoA-Dehydrogenase-Deficiency (LCHADD) or Very-Long-Chain-3-Hydroxy-Acyl-CoA-Dehydrogenase-Deficiency (VLCADD) frequently present with hypertrophic cardiomyopathy or muscle weakness which is caused by the accumulation of fatty acid metabolites due to...

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Detalhes bibliográficos
Publicado no:Sci Rep
Main Authors: Hagenbuchner, Judith, Scholl-Buergi, Sabine, Karall, Daniela, Ausserlechner, Michael J.
Formato: Artigo
Idioma:Inglês
Publicado em: Nature Publishing Group UK 2018
Assuntos:
Acesso em linha:https://ncbi.nlm.nih.gov/pmc/articles/PMC5818531/
https://ncbi.nlm.nih.gov/pubmed/29459657
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1038/s41598-018-21519-2
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