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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… Expand
National Institutes of Health

Related topics

Related topics

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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Highly Cited
2005
Highly Cited
2005
Effective gene therapy for an inherited CNS disease in a large animal model
Genetic diseases affecting the brain typically have widespread lesions that require global correction. Lysosomal storage diseases… Expand
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Highly Cited
2005
Highly Cited
2005
Biological properties of D- and L-1-deoxyazasugars.
L-Enantiomers of 1-deoxynojirimycin (DNJ), 1-deoxymannojirimycin (manno-DNJ), 1-deoxyallonojirimycin (allo-DNJ), 1… Expand
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Highly Cited
2002
Highly Cited
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… Expand
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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… Expand
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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… Expand
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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…
Current evidence indicates that the trimming of mannosyl residues from intermediates in the biosynthesis of the N-linked… Expand
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Highly Cited
1982
Highly Cited
1982
Chorion biopsy in early pregnancy: A method of early prenatal diagnosis for inherited disorders
Chorion biopsy was performed in 165 cases at 6–12 weeks of pregnancy, following an ultrasonic or embryo‐fetoscopic chorion… Expand
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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… Expand
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Highly Cited
1978
Highly Cited
1978
Carbohydrate structure of vesicular stomatitis virus glycoprotein.
The carbohydrate structure of the membrane glycoprotein of vesicular stomatitis virus (New Jersey serotype) has been determined… Expand
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Highly Cited
1973
Highly Cited
1973
A mannose-containing trisaccharide isolated from urines of three patients with mannosidosis.
Abstract The urine from three patients with mannosidosis was found to contain abnormally high amounts of mannose-containing… Expand
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