title: Phenylalanine metabolism and intellectual functioning among carriers of phenylketonuria and hyperphenylalaninaemia.
abstract: All members of 63 families who had phenylketonuric or hyperphenylalaninaemic offspring received a phenylalanine tolerance test and an individual intelligence test. Parent carriers (heterozygotes, n=115) displayed a significant relationship (canonical correlation R=0.75, P is less than 0.05) between their ability to metabolise phenylalanine and their intellectual strengths and weaknesses. Mothers of hyperphenylalaniaemic children did not show this relationship. The number of carrier children (n=40) was too small for multivariate or sex analysis, but significant bivariate correlations were found for this group.
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006579:Heterozygote; D006801:Humans; D007360:Intelligence; D008297:Male; D010649:Phenylalanine / Q000097:blood / Q000378:metabolism*; D010661:Phenylketonurias / Q000235:genetics* / Q000378:metabolism; D014443:Tyrosine / Q000097:blood; D014888:Wechsler Scales
chemical_list: D014443:Tyrosine; D010649:Phenylalanine