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The cause of type 1PKU is the fact that the phenylalanine hydroxylase enzyme is not present or that it is defective. The cause pf PKU type 2, which hardly occurs is to do with the tetrahydrobioprotein co factor. PKU causes the accumulation of phenylalanine in all of body's fluids. This accumulation is due to the fact phenylalanine is not able to convert into the tyrosine. According to Biochemistry, 5th Edition (2002), J.M. Berg, J. L. Tymoczko and L. Stryer, Ch 23 Protein Turnover and Amino Acid Catabolism, "three quarters of phenylalanine converted into Tyrosine and the other quarter incorporated into proteins...
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