Crib death of infants when fasting (i.e., when sleeping and…
Crib death of infants when fasting (i.e., when sleeping and not eating – therefore a low blood glucose condition) is also known as sudden infant death syndrome (SIDS). SIDS was often blamed on lack of proper care by the parents of the child. It is now known that a significant number of SIDS cases are caused by a genetic defect in the gene that codes for fatty acyl-CoA dehydrogenase, the enzyme that catalyzes the first step in fatty acid β-oxidation (see question 34 for this reaction). If this enzyme is defective, energy from fatty acids is not available and, in addition, gluconeogenesis is inhibited resulting in severe and life-threatening hypoglycemia (low blood glucose). Why the hypoglycemia?
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