The activity of acid hydrolases on 13 substrates has been measured in the liver of normal
human subjects and of 32 patients with various forms of mucopolysaccharidoses. The most …

Early effects of gentamicin, tobramycin, and amikacin on the human kidney Page 1 Kidney
International, Vol. 25 (1984), PP. 643—652 CLINICAL INVESTIGATION Early effects of …

Anatomopathological studies are reported in a new case of Pompe's disease (glycogenosis
type II). The topography of the selective neuronal involvement is again stressed and more …

To evaluate the role of dietary polyamines in maturation of the rat small intestine, spermine
was given orally twice daily to suckling pups from day 10 to day 14 postpartum at different …

Gentamicin is an antibiotic that exhibits a broad spectrum of activity and is particularly
valuable in severe sepsis. Its use is, however, restricted because of the development of …

Lafora bodies were studied in a parietal cortex biopsy of a patient suffering from progressive
myoclonic epilepsy, both histochemically (with the optical microscope) and in the electron …

The inhibitory effects of valproate (VPA) and nine of its metabolites on mitochondrial
oxidations have been investigated. Valproate, 4-ene-VPE, 2, 4-diene-VPA and 2 …

We describe an infant girl with a clinical, chemical, and pathologic syndrome remarkably
similar to Zellweger cerebrohepatorenal syndrome but whose liver parenchymal cells …

Dicarboxylic acids are products of the omega-oxidation of monocarboxylic acids. We
demonstrate that in rat liver dicarboxylic acids (C5-C16) can be converted into their CoA …

Publisher Summary This chapter discusses the enzymes of glycogen degradation involved
in biopsy material. The determination of enzymatic activities in human tissues requires the …