GCDH and hyperinsulinemic hypoglycemia, familial, 4: The best example of this is glutaryl-CoA dehydrogenase deficiency, which leads to accumulation of glutaryl-CoA, causing increased lysine glutarylation via chemical acylation, ultimately leading to reduced function of other mitochondrial proteins such as carbamoyl phosphate synthase-1 (Cps1) in the urea cycle [29].