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Mannosidase Deficiency Diseases

Known as: Deficiency Syndromes, Mannosidase, Mannosidosis, Deficiency Syndrome, Mannosidase 
A rare autosomal recessive lysosomal storage disease characterized by a deficient activity of the enzymes alpha-D-mannosidase or beta-mannosidase… 
National Institutes of Health

Related topics

Related topics

12 relations

Broader (2)

Enzyme DeficiencyLysosomal Storage Diseases
Hereditary DiseasesIn BloodMannosidaseMental Retardation

Narrower (1)

alpha-Mannosidosis

Papers overview

Semantic Scholar uses AI to extract papers important to this topic.
Highly Cited
2005
Highly Cited
2005
Neurocognitive and Psychotiform Behavioral Alterations and Enhanced Hippocampal Long-Term Potentiation in Transgenic Mice Displaying Neuropathological Features of Human α-Mannosidosis
Mice with α-mannosidase gene inactivation provide an experimental model for α-mannosidosis, a lysosomal storage disease with… 
Highly Cited
1982
Highly Cited
1982
alpha-D-Mannosidases of rat liver Golgi membranes. Mannosidase II is the GlcNAcMAN5-cleaving enzyme in glycoprotein biosynthesis and mannosidases Ia and IB are the enzymes converting Man9 precursors…
Highly Cited
1981
Highly Cited
1981
Caprine beta-mannosidosis. Inherited deficiency of beta-D-mannosidase.
1980
1980
A 500 MHz 1H NMR study of urinary oligosaccharides from patients with mannosidosis
Highly Cited
1976
Highly Cited
1976
Increased levels of sialic acid associated with a sialidase deficiency in I-cell disease (mucolipidosis II) fibroblasts.
Highly Cited
1976
Highly Cited
1976
Lipid-saccharide intermediates in glycoprotein biosynthesis. II. Studies on the structure of an oligosaccharide-lipid from thyroid.
Structural studies have been performed on an oligosaccharide-lipid from thyroid believed to be an intermediate in glycoprotein… 
Highly Cited
1974
Highly Cited
1974
Effect of Streptozotocin Diabetes on Acid Phosphatase and Selected Glycosidase Activities of Serum and Various Rat Organs
Summary The activities of the lysosomal hydrolases α-l-fucosidase, α-d-mannosidase, β-d-galactosidase, and N-acetyl-β-d-glucosa… 
Highly Cited
1973
Highly Cited
1973
A mannose-containing trisaccharide isolated from urines of three patients with mannosidosis.
Highly Cited
1973
Highly Cited
1973
A New α-d-Mannosidase Occurring in Golgi Membranes
Highly Cited
1973
Highly Cited
1973
MANNOSIDOSIS: CLINICAL, FINE‐STRUCTURAL AND BIOCHEMICAL FINDINGS IN THREE CASES
Three boys, 4, 5, and 10 years old, with psycho‐motor retardation, slightly gargoyle‐like faces and recurrent infections were… 
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