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… (More)
National Institutes of Health

Related topics

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12 relations

Broader (2)

Enzyme DeficiencyLysosomal Storage Diseases
Hereditary DiseasesIn BloodMannosidaseMental Retardation
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Narrower (1)

alpha-Mannosidosis

Papers overview

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2009
2009
Cloning, expression in Pichia pastoris, and characterization of a thermostable GH5 mannan endo-1,4-β-mannosidase from Aspergillus niger BK01
BACKGROUND Mannans are key components of lignocellulose present in the hemicellulosic fraction of plant primary cell walls… (More)
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2006
2006
The affinity of the FimH fimbrial adhesin is receptor-driven and quasi-independent of Escherichia coli pathotypes
Type-1 fimbriae are important virulence factors for the establishment of Escherichia coli urinary tract infections. Bacterial… (More)
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2002
2002
Germ cell survival through carbohydrate-mediated interaction with Sertoli cells.
Spermatogenesis is a precisely regulated process in which the germ cells closely interact with Sertoli cells. The molecular basis… (More)
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Review
1999
Review
1999
Bone marrow transplantation as effective treatment of central nervous system disease in globoid cell leukodystrophy, metachromatic leukodystrophy, adrenoleukodystrophy, mannosidosis, fucosidosis…
Over 400 patients with lysosomal and peroxisomal storage diseases have received hematopoietic stem cell transplantation from… (More)
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1999
1999
A lysosomal storage disease induced by Ipomoea carnea in goats in Mozambique.
A novel plant-induced lysosomal storage disease was observed in goats from a village in Mozambique. Affected animals were ataxic… (More)
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Highly Cited
1995
Highly Cited
1995
Cloning and expression in Saccharomyces cerevisiae of a Trichoderma reesei beta-mannanase gene containing a cellulose binding domain.
beta-Mannanase (endo-1,4-beta-mannanase; mannan endo-1,4-beta-mannosidase; EC 3.2.1.78) catalyzes endo-wise hydrolysis of the… (More)
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1991
1991
Neuroaxonal dystrophy in neuronal storage disorders: Evidence for major GABAergic neuron involvement
The formation of focal granular enlargements within axons (axonal spheroids or "torpedoes"; neuroaxonal dystrophy) is a well… (More)
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1987
1987
Cloning and heterologous expression of glycosidase genes from Saccharomyces cerevisiae.
Genomic clones were isolated that code for three glycosidases proposed to be involved in the catabolism of cell wall components… (More)
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Highly Cited
1980
Highly Cited
1980
A light and electron microscopic study of mannosidosis.
The present work investigated the light and electron microscopic changes in hypertrophied gingiva in a patient with mannosidosis… (More)
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Highly Cited
1980
Highly Cited
1980
Inhibition of lysosomal alpha-mannosidase by swainsonine, an indolizidine alkaloid isolated from Swainsona canescens.
An indolizidine alkaloid (swainsonine) was isolated from the plant Swainsona canescens. Swainsonine is a specific and potent… (More)
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