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Description
Mitochondrial dysfunction in mut methylmalonic acidemia
The aforementioned information leads to the conclusion that sample and standard solutions are consistent up to 78 hours and 68 hours, respectively, when stored at room temperature
Due to the lack of an acyl-CoA transporter in the mitochondrial inner membrane, the acyl group is transferred to the shuttle molecule carnitine for translocation into the matrix (Figure 1)
In the setting of an acute overdose, stop the valproic acid and give the patient L-carnitine
[PubMed] [Google Scholar] 13.Gilgore S.G., DeFelice S.L
