ed 3-methyglutaconic acid, 3-methylglutaric acid, and 2-ethyl-3-hydroxy-propionic acid Long- chain fatty acids, acyl-camitines, lysosomal hydmlases, li-galactosidase, 13-glucumnidase, sphingornyelinase, glucocerebrosidase, a- t•iduronidase, and a -glucosaminidase were normal Pressors were required for hypo- tens