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Further experiments demonstrated that CPT1A is mainly located in the mitochondria of living cells, which can be directly and conveniently observed by confocal microscopy (Fig

[PubMed: 30689447] (70 year old woman developed seizures within 30 minutes after mistakenly taking an industrial form of ALA [4500 mg] with metabolic acidosis, lactic acidosis [14.9 mmoL], decrease in platelets [140,000 decreasing to 73,000/uL], and hemodynamic instability, responding to supportive therapy within several days)

1 mM/l glutamate de sodium et 10 mM/l BES (acide N,N-bis[2-hydroxythyl]-2-aminothanesulfonique).The nerve-muscle preparation is bathed in a modified Liley solution suitably oxygenated (95% O and 5% CO) at 37 o C, (137 mM / l NaCl

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