ACADS and hyperinsulinemic hypoglycemia, familial, 4: Edhager et al employed a quantitative mitochondrial proteomic approach using fibroblast cell line pathogenic variants in ACADS, encoding a mitochondrial flavoprotein, to identify over 300 interacting proteins, and map protein perturbations to mitochondrial pathways in order to differentiate between mild and severe ACADS deficiency, in addition to confirming a severe reduction in the SCAD protein [90].