Add to ORKGIt is not yet clear whether lysosomal enzymes act only in degenerative processes, in response to noxious stimuli, or whether they act in the normal degradative pro-cesses of turnover. In support of the latter belief is the observation that specific lyso-somal enzymes are absent (or highly deficient) in certain genetic lesions characterize
A method is described for the preparation of a [beta]-galactosidase which acts on the brain lipid, c...
A deficiency of lysosomal sialidase has recently been identified among a group of disorders which ha...
A lysosomal preparation, obtained from brain homogenate of 17-day-old C57BL mice by centrifugation o...
Four enzyme assays were carried out with brains from rats of age 4 days to about 320 days. The enzym...
(1) The brains of 17-day-old quaking and jimpy mice were compared with those of the corresponding no...
Glycosyl hydrolases (GHs) are carbohydrate-active enzymes that hydrolyze a specific β-glycosidic bon...
textabstractThe experimental work presented in this thesis deals with the genetic and molecular bas...
Glycosphingolipids, comprising a ceramide lipid backbone linked to one/more saccharides, are particu...
Glycosphingoid bases are elevated in inherited lysosomal storage disorders with deficient activity o...
textabstractLysosomal B-galactosidase is the glycosidase, that cleaves B-linked galactosyl mmenes fr...
The specific activities of five glycohydrolases of lysosomal origin (\u3b2-N-acetylglucosaminidase, ...
Lysosomal storage diseases are disorders caused by deficiencies of enzymes responsible for the degra...
textabstractSince the discovery of the lysosome as a distinct subcellular compartment important for ...
textabstractThe lysosome represents a well characterized, membrane-contained intracellular digestive...
The balance between survival and death in many cell types is regulated by small changes in the intra...
A method is described for the preparation of a [beta]-galactosidase which acts on the brain lipid, c...
A deficiency of lysosomal sialidase has recently been identified among a group of disorders which ha...
A lysosomal preparation, obtained from brain homogenate of 17-day-old C57BL mice by centrifugation o...
Four enzyme assays were carried out with brains from rats of age 4 days to about 320 days. The enzym...
(1) The brains of 17-day-old quaking and jimpy mice were compared with those of the corresponding no...
Glycosyl hydrolases (GHs) are carbohydrate-active enzymes that hydrolyze a specific β-glycosidic bon...
textabstractThe experimental work presented in this thesis deals with the genetic and molecular bas...
Glycosphingolipids, comprising a ceramide lipid backbone linked to one/more saccharides, are particu...
Glycosphingoid bases are elevated in inherited lysosomal storage disorders with deficient activity o...
textabstractLysosomal B-galactosidase is the glycosidase, that cleaves B-linked galactosyl mmenes fr...
The specific activities of five glycohydrolases of lysosomal origin (\u3b2-N-acetylglucosaminidase, ...
Lysosomal storage diseases are disorders caused by deficiencies of enzymes responsible for the degra...
textabstractSince the discovery of the lysosome as a distinct subcellular compartment important for ...
textabstractThe lysosome represents a well characterized, membrane-contained intracellular digestive...
The balance between survival and death in many cell types is regulated by small changes in the intra...
A method is described for the preparation of a [beta]-galactosidase which acts on the brain lipid, c...
A deficiency of lysosomal sialidase has recently been identified among a group of disorders which ha...
A lysosomal preparation, obtained from brain homogenate of 17-day-old C57BL mice by centrifugation o...