In Phenylketonuria: The Importance of the Structural Integrity of Phenylalanine Hydroxylase paper the reviewer talks about Phenylketonuria being a recessive disease that is hereditary impacting the metabolic function of the phenylalanine hydroxylase enzyme and neurological mechanisms. A comparison between the mutated enzyme and the normal enzyme called the wild type is made to fully understand how the enzyme is supposed to work and what it causes if there is a malfunction. An interesting point made is that the enzymes function varies on the cell and how the organism uses it. The review focused on how phenylalanine functions, is activated, and regulated in mammalian eukaryotes. The elevated levels phenylalanine seems to cause the neurological problems due to the binding of the enzyme and how much phenylalanine is leftover flowing in the bloodstream after crossing the blood-brain…show more content… The high levels also impact the neurotransmitter pathway and overall reaction balance. This malfunction can be caused by possible factors the phenylalanine being toxic to the cell and the over binding to all receptors present. A definite cause seems unknown. The structure of this enzyme is interconnected to the function for if it has the wrong folding or just a mutation the overall faction is altered. The activation of the enzyme is controlled through phosphorylation. Phosphorylating can be used by the cell as an on and of switch on phenylalanine. BH4 is the known regulator of the enzyme, where it allows the molecule rotate and become inactive through a confirmation change. However, the importance, of this mechanism to properly function, is to maintain balance how much phenylalanine is present in the bloodstream and how much it binds to the enzyme so that the rest of the reactions can be controlled as
