Fucosidosis is a rare, autosomal recessive, lysosomal storage disorder caused by a severe
deficiency of alpha-L-fucosidase in all tissues. We have conducted a review of fucosidosis …

A wide variety of inherited lysosomal hydrolase deficiencies have been reported in animals
and are characterized by accumulation of sphingolipids, glycolipids, oligosaccharides, or …

Carbohydrate Chemistry and Glycobiology have witnessed a rapid expansion during the last
few years with the development of numerous new, imaginative and efficient syntheses which …

There are more than 40 different forms of inherited lysosomal storage diseases (LSDs)
known to occur in humans and the aggregate incidence has been estimated to approach 1 …

Lysosomal storage diseases (LSDs) are inherited metabolic disorders caused by deficient
activity of a single lysosomal enzyme or other defects resulting in deficient catabolism of …

Krabbe disease or globoid cell leukodystrophy (GLD) is an autosomal recessive disorder
resulting from the defective lysosomal hydrolysis of specific galactolipids found primarily in …

Oral administration of the long-acting somatostatin analogue, SMS 201-995 (Sandoz), was
assessed in five patients with active acromegaly, four of whom had not responded to …

A polysystemic disorder was observed in three related English Springer Spaniel dogs that
demonstrated regurgitation from an early age, slowly progressive temporal muscle atrophy …

Ceroid lipofuscinosis was diagnosed by hlstopathological and histochemical findings in 17
related border collie dogs and by clinical signs in 6 of their litter mates. Behavioural …

Cortical pyramidal neurons in several types of neuronal storage diseases have been shown
by Golgi staining to sprout axon hillock-associated dendritic processes. Based on the …