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Mutation update on ACAT1 variants associated with mitochondrial acetoacetyl‐CoA thiolase (T2) deficiency

Mitochondrial acetoacetyl‐CoA thiolase (T2, encoded by the ACAT1 gene) deficiency is an inherited disorder of ketone body and isoleucine metabolism. It typically manifests with episodic ketoacidosis. The presence of isoleucine‐derived metabolites is the key marker for biochemical diagnosis. To date,...

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Foilsithe in:Hum Mutat
Main Authors: Abdelkreem, Elsayed, Harijan, Rajesh K., Yamaguchi, Seiji, Wierenga, Rikkert K., Fukao, Toshiyuki
Formáid: Artigo
Teanga:Inglês
Foilsithe: John Wiley and Sons Inc. 2019
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Rochtain Ar Líne:https://ncbi.nlm.nih.gov/pmc/articles/PMC6790690/
https://ncbi.nlm.nih.gov/pubmed/31268215
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1002/humu.23831
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