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Natural history, outcome, and treatment efficacy in children and adults with glutaryl-CoA dehydrogenase deficiency

PMID: 32436359 Biochem PharmacolIsoalantolactone targets NLRP3 to disrupt NLRP3-NEK7 interaction and suppress inflammasome activation

The patient was continued on a 3-day course of diazepam 5 mg IM BID, maintenance fluids (dextrose 5% in 0.45% normal saline with 20 meq of potassium infusing at 70 mL/h for 4 days), and a 5 day course of benztropine 2 mg PO BID

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In contrast, neurons prioritize maintaining ion gradients for electrical signaling, with mitochondria concentrated near synapses to support neurotransmitter release