beta-Mannosidase

An enzyme that catalyzes the hydrolysis of terminal, non-reducing beta-D-mannose residues in beta-D-mannosides. The enzyme plays a role in the lysosomal degradation of the N-glycosylprotein glycans. Defects in the lysosomal form of the enzyme in humans result in a buildup of mannoside intermediate metabolites and the disease BETA-MANNOSIDOSIS.

Also Known As:

Networked: 59 relevant articles (1 outcomes, 2 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Beccari, T: 3 articles (06/2009 - 12/2000)
2.	Levade, Thierry: 2 articles (01/2009 - 01/2009)
3.	Labauge, Pierre: 2 articles (01/2009 - 01/2009)
4.	de Champfleur, Nicolas: 2 articles (01/2009 - 01/2009)
5.	Castelnovo, Giovanni: 2 articles (01/2009 - 01/2009)
6.	Sabourdy, Frédérique: 2 articles (01/2009 - 01/2009)
7.	Renard, Dimitri: 2 articles (01/2009 - 01/2009)
8.	Jackson, M E: 2 articles (12/2004 - 07/2003)
9.	Orlacchio, A: 2 articles (06/2001 - 12/2000)
10.	Balducci, C: 1 article (06/2009)

Related Diseases

1.	Gingivitis 01/01/1992 - "Only the specific activities (as units of enzyme activity per min per microliter of GCF) of beta-mannosidase and hexosaminidase A were significantly different between the groups being greater in the gingivitis group. " 01/01/1992 - "In contrast, the total beta-mannosidase and hexosaminidase A activities were significantly greater in the gingivitis group, while there was no significant difference in the total alpha-mannosidase activity between the groups. "
2.	beta-Mannosidosis (beta-Mannosidase Deficiency) 07/01/2003 - "Morphological and biochemical studies of human beta-mannosidosis: identification of a novel beta-mannosidase gene mutation." 04/10/1982 - "These studies suggest that goat liver contains both lysosomal beta-mannosidase (acidic form; deficient in beta-mannosidosis) and nonlysosomal beta-mannosidase (neutral) activity." 01/01/2009 - "beta-Mannosidosis (OMIM 248510) is a rare inborn lysosomal storage disorder caused by the deficient activity of beta-mannosidase, an enzyme encoded by a single gene (MANBA) located on chromosome 4q22-25. " 01/01/2009 - "Beta-mannosidosis (OMIM 248510) is an inborn lysosomal storage disorder caused by deficiency of beta-mannosidase activity. " 08/01/2008 - "We propose that the milder phenotype described in some beta-mannosidosis patients with missense mutations in the MANBA gene is not due to residual beta-mannosidase activity, but rather caused by epigenetic and/or environmental factors."
3.	Lysosomal Storage Diseases (Lysosomal Storage Disease) 07/01/2003 - "beta-Mannosidosis (MANB1; OMIM248510), first reported in humans in 1986, is a rare hereditary lysosomal storage disease caused by a deficiency of the enzyme beta-mannosidase. " 12/01/2002 - "Beta-mannosidosis is an autosomal recessive lysosomal storage disease resulting from a deficiency of the lysosomal enzyme beta-mannosidase. " 01/01/1998 - "Human beta-mannosidosis is an autosomal recessive, lysosomal storage disease caused by a deficiency of the enzyme beta-mannosidase. " 01/01/1991 - "A diagnosis of beta-mannosidosis, a lysosomal storage disease caused by a deficiency of beta-mannosidase, was made in 12 purebred Salers calves. " 04/01/1983 - "The assay described can be used to evaluate plasma beta-mannosidase measurements as a test for detecting carriers of caprine beta-mannosidosis, a newly described lysosomal storage disease."
4.	Mannosidase Deficiency Diseases (Mannosidosis) 01/01/1990 - "Interest in using caprine beta-D-mannosidosis as a model to evaluate bone marrow transplantation in the treatment of human lysosomal storage disorders provided the stimulus for characterization of beta-D-mannosidase in selected goat tissues and induction of hemopoietic chimerism in the goat. " 03/01/1997 - "This clan includes five enzymes implicated in lysosomal storage diseases: beta-glucuronidase (Sly disease), beta-glucocerebrosidase (Gaucher disease), beta-galactosidase (Landing disease and Morquito type B disease), beta-mannosidase (mannosidosis) and alpha-L-iduronidase (Hurler-Scheie disease). "
5.	Body Weight (Weight, Body) 06/01/2005 - "However, the addition of beta-mannanase significantly increased average egg production and egg mass of hens fed the low-energy diet from wk 5 to 8. There were no significant differences in feed intake, egg specific gravity, egg weight, mortality, body weight, and body weight variability among the 3 dietary treatments. " 12/01/2004 - "An experiment was designed to assess the effects of graded levels of beta-mannanase on performance and body weight uniformity of male broilers provided with diets based on corn and soybean meal and devoid of antibiotic growth promoters or coccidiostats. "

Related Drugs and Biologics

1.	alpha-Mannosidase (LAMAN)
2.	Hexosaminidase A (Hex A)
3.	Oligosaccharides
4.	Glycoproteins (Glycoprotein)
5.	Glucuronidase
6.	Enzymes
7.	beta-N-Acetylhexosaminidases
8.	beta-Galactosidase (Lactaid)
9.	beta-Mannosidase
10.	Complementary DNA (cDNA)

Related Therapies and Procedures

1.	Bone Marrow Transplantation (Transplantation, Bone Marrow)