Children with extreme forms of PKU often
have blond hair and reduced body pigment.
This is because the absence of PAH is a
metabolic block that prevents conversion of
phenylalanine into tyrosine, which is the
precursor of the pigment melanin. The relationship
between severe mental retardation
and decreased pigmentation in PKU makes
sense only if one knows the metabolic connections
among phenylalanine, tyrosine,
and melanin. If these connections were not
known, the traits would seem completely
unrelated. PKU is not unusual in this regard.
Many mutant genes affect multiple
traits through their secondary or indirect
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