Development of Adenosine 5'-Triphosphate Sulfurylase and Adenosine Phosphosulfate Kinase in Rat Cerebrum and Liver

Abstract

The development of the enzymes of sulfate activation, ATP-sulfurylase and APS-kinase has been studied in rat cerebrum and, for comparison, in rat liver as well. Cerebrum contains a species of ATP-sulfurylase which can be inhibited by phenylalanine. The activity of this form of ATP-sulfurylase is high in oligodendroglial cells and parallels the rate of myelination.

White matter disturbances in phenylketonuria: Possible underlying mechanisms

“…Transgenic mice lacking enzymes for the synthesis of sulfatides or galactoceramides have severe motor symptoms, thinner myelin sheaths and abnormal nodal/paranodal structures (Marcus et al, 2006;Schmitt et al, 2015). In this regard, the brain of HPA animal models shows low levels of ceramides and sulfatides and low enzyme activity of galactosylceramide sulfotransferase and adenylyltransferase sulfate (Burri, Matthieu, et al, 1990;Burri, Steffen, et al, 1990;Hommes, 1991;Matsuo, Moss, & Hommes, 1987). Impaired myelination in PKU was subsequently associated with the inhibition of fatty acid synthase and citrate synthase in the presence of phenylpyruvate in rat brain (Land & Clark, 1973).…”

Dietary Phenylalanine and Brain Function

“…The phenylalanine-sensitive ATP-sulfurylase is most active in those areas of the brain most heavily affected by the PKU condition (Matsuo and Hommes 1987), it is active in oligodendroglial cells, and its activity closely parallels the rate of myelination (Matsuo et al 1987b). These are necessary requirements for the involvement of this enzyme in the etiology of brain dysfunction in PKU.…”