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Zambrano S, Blanca AJ, Ruiz-Armenta MV, Miguel-Carrasco JL, Arvalo M, Mate A, et al

Newborn screening: a disease-changing intervention for glutaric aciduria type 1

L-carnitine deficiency severely impairs the mitochondrial import of long-chain acyl-CoAs, directly limiting the acetyl-CoA pool required for ketogenesis ( -oxidation activity ( Figure 2 The preterm brain exhibits extraordinarily high energy demands to fuel rapid growth and development, demands which can surpass the capacity of glucose supply alone

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