Results 151 to 160 of about 7,983 (189)
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Preferential Inhibition of Lysosomal Beta-Mannosidase by Sucrose
Enzyme, 1990The lysosomal storage disease beta-mannosidosis, described in both goats and humans, can be detected by measuring a deficiency in hydrolysis of the fluorogenic substrate 4-methylumbelliferyl-beta-D-mannoside. An inhibitor of guinea pig beta-mannosidase (beta-man) activity was detected when tissue was homogenized in phosphate-buffered-saline (pH 7.4 ...
N R, McCabe, W, Biliter, G, Dawson
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5605797 Bovine beta-mannosidase gene and methods of use
Biotechnology Advances, 1997exaly +2 more sources
ChemInform Abstract: A New Strong Inhibitor of Beta‐Mannosidase.
ChemInform, 1998AbstractChemInform is a weekly Abstracting Service, delivering concise information at a glance that was extracted from about 100 leading journals. To access a ChemInform Abstract of an article which was published elsewhere, please select a “Full Text” option. The original article is trackable via the “References” option.
H. THERISOD, Y. LETOURNEUX, M. THERISOD
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Bovine β-mannosidase deficiency
Biochemical and Biophysical Research Communications, 1990A fatal inherited glycoprotein storage disorder is described in Salers cattle which affects both sexes. Affected calves are unable to stand at birth, have a marked intention tremor, markedly enlarged kidneys, decreased white matter in all areas of the brain, and cytoplasmic vacuolation in multiple cell types of multiple tissues with nervous, renal ...
L, Bryan +3 more
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Human β-Mannosidase Deficiency
New England Journal of Medicine, 1986LYSOSOMAL disorders in humans due to deficiencies of lysosomal enzymes or effector proteins (activator or protector proteins) have been described for most steps in the catabolism of glycoproteins, ...
D A, Wenger +3 more
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Human chorionic β‐mannosidase: Comparison with β‐mannosidase from human cultured fibroblasts
Prenatal Diagnosis, 1992AbstractThe conditions for assay of β‐mannosidase activity in human chorionic villi (CV) were studied using the fluorogenic substrate 4‐methylumbelliferyl‐β‐D‐mannopyranoside. A comparison of the biochemical properties of the CV β‐mannosidase with those of the enzyme from human cultured fibroblasts showed their similarity.
N A, Petushkova +2 more
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β‐Mannosidase in human leukocytes and fibroblasts
Journal of Inherited Metabolic Disease, 1984AbstractIn human leukocytes and fibroblasts β‐mannosidase activity has a unimodal pH optimum (4.0–4.5). Markedly reduced activity is found in I‐cell disease. Normal activities in human fibroblasts are ten times higher than in the goat, in which species a deficiency disease has been described.
R S, Panday +3 more
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beta-Mannosidase deficiency in Anglo Nubian goats.
Australian veterinary journal, 1982Two newly-born Anglo Nubian kids with clinical evidence of a severe neurological condition were examined. Pathological examination revealed cytoplasmic vacuolation in neural tissue and in localized areas in the kidney, pancreas and lymphoid tissue. Biochemical investigations demonstrated a deficiency of beta-mannosidase in tissues and blood serum.
P F, Healy +3 more
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