Which organic compound is implicated in Phenylketonuria?

Phenylketonuria (PKU) is a genetic disorder caused by the deficiency of the enzyme phenylalanine hydroxylase. This enzyme is crucial for the metabolism of phenylalanine, an amino acid that is found in many protein-containing foods and is essential for normal growth and development. In individuals with PKU, the inability to metabolize phenylalanine leads to its accumulation in the body, which can cause significant neurological impairments and other serious health problems if not managed properly through dietary restrictions.

The correct answer, phenylalanine, is directly related to the condition as it is the compound that accumulates due to the enzyme deficiency. Managing PKU typically involves a low-phenylalanine diet to prevent toxic buildup and protect brain function, making understanding its role crucial for the management of the disorder.

The other compounds listed do not have a direct association with PKU. Cholesterol and maltose serve different roles in the body related to lipid metabolism and carbohydrates, respectively, while ribose, a sugar involved in nucleic acid synthesis, is not implicated in the metabolic pathway associated with PKU. Thus, phenylalanine is clearly central to the pathophysiology of this disorder.