Hydroxylamine and a 32P-Labelled Intermediate in Sodium-plus-Potassium Ion-Activated Adenosine Triphosphatase

Abstract

despite the obvious degeneration of white matter in globoid leucodystrophy, other transferases were within normal limits in brain from a globoid-leucodystrophy patient in previous studies (e.g. UDP glucose-glycogen glucosyltransferase) (Austin et al. 1963). An enzymic disorder of sulphate conjugation has not hitherto been reported. The expected result in vivo of a cerebroside sulphotransferase activity deficiency would be a deficiency of sulphatides. Because sulphatides are normally most concentrated in myelin, a deficiency of sulphatides could interfere with the proper development of myelin in this human disease.