This study examined motor control in 61 early and continuously treated patients with phenylketonuria (PKU) and 69 control participants, aged 7 to 14 years. The pursuit task demanded concurrent planning and execution of unpredictable movements, whereas the tracking task required a highly automated circular movement that could be planned in advance. PKU patients showed significantly poorer motor ...

Moncrieff and Wilkinson (1961) and Brimblecombe, Blainey, Stoneman and&-Wood (1961) have described the development of a severe extensive skin eruption in babies fed on the low-phenvlalanine food 'minafen' (Trufood Ltd.). More recently, two further phenylketonuric children have been seen at Great Ormond Street with bizarre rashes. One, a baby, developed a rash similar to that described by Moncri...

This article provides a targeted review of the scientific literature on the effects of experience on early brain and behavioral development and later outcome as it pertains to risk for some forms of child psychopathology. It is argued that ample evidence exists indicating that the prenatal and early postnatal years likely represent a sensitive period with respect to the effects of stress on the...

A South Wales gypsy kindred of Romany origin had a high incidence of phenylketonuria along with other recessively inherited disorders. There was a high degree of consanguinity (F = 0-017) with an excess of non-specific mental subnormality among known consanguineous matings. Phenylketonuria and a number of other recessively inherited disorders have been recorded from other Romany gypsy populatio...

A 1993 MRC working group on phenylketonuria suggested standardising blood phenylalanine measurements by taking blood samples at the same time each day. Since it is not known how representative of a 24 hour period a single phenylalanine concentration is, the aim of this study was to investigate the 24 hour variability of plasma phenylalanine in well controlled children with phenylketonuria. Sixt...

DNA gyrase is the only topoisomerase capable of introducing (-) supercoils into relaxed DNA. The C-terminal domain of the gyrase A subunit (GyrA-CTD) and the presence of a gyrase-specific 'GyrA-box' motif within this domain are essential for this unique (-) supercoiling activity by allowing gyrase to wrap DNA around itself. Here we report the crystal structure of Xanthomonas campestris GyrA-CTD...

The development of a practical screening procedure for phenylketonuria and the improvement in methods of chemical analysis have led to a realization that Folling's (1934) disease of phenylketonuria is not a single entity. In this commentary, the current view on some aspects of phenylketonuria will be reviewed and the problems illustrated by experience gained in the Phenylketonuria Clinic at the...