A Teacher's Guide to PKU
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Phenylketonuria (or PKU) is an inherited disorder of protein metabolism. Children with PKU do not have a functioning enzyme to metabolize or break down amino acid call phenylalanine (PHE for short), which is found in all food proteins. Protein in foods is important for building and repairing the body's tissues. Amino acids are often called the "building blocks" of protein. Twenty-two amino acids can be joined together in various combinations to form all the different kinds of proteins in foods. Enzymes are special substances in the body which work to separate the amino acids in food proteins and recombine them to form the different proteins which the body needs.