Phenylalanine hydroxylase is the rate-limiting enzyme of the metabolic pathway In biochemistry, metabolic pathways are series of chemical reactions occurring within a cell. In each pathway, a principal chemical is modified by chemical reactions. Enzymes catalyze these reactions, and often require dietary minerals, vitamins, and other cofactors in order to function properly. Because of the many chemicals that may be involved, which degrades excess phenylalanine.
The other substrates in the reaction are molecular oxygen and tetrahydrobiopterin US FDA:link. Tetrahydrobiopterin is a member of the group of redox biochemicals known as pteridines. PAH is the gene A gene is the basic unit of heredity in a living organism. All living things depend on genes. Genes hold the information to build and maintain an organism's cells and pass genetic traits to offspring. A modern working definition of a gene is "a locatable region of genomic sequence, corresponding to a unit of inheritance, which is associated that encodes for phenylalanine hydroxylase. It was the research on phenylalanine hydroxylase by Seymour Kaufman that led to the discovery of tetrahydrobiopterin as a biological cofactor.[1]
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