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: 64 relevant articles (1 outcomes, 3 trials/studies)

Relationship Network

Bio-Agent Context: Research Results

Experts

1.	Beccari, T: 3 articles (06/2009 - 12/2000)
2.	Castelnovo, Giovanni: 2 articles (09/2009 - 01/2009)
3.	Labauge, Pierre: 2 articles (09/2009 - 01/2009)
4.	Levade, Thierry: 2 articles (09/2009 - 01/2009)
5.	Renard, Dimitri: 2 articles (09/2009 - 01/2009)
6.	Sabourdy, Frédérique: 2 articles (09/2009 - 01/2009)
7.	de Champfleur, Nicolas: 2 articles (09/2009 - 01/2009)
8.	Jackson, M E: 2 articles (12/2004 - 07/2003)
9.	Orlacchio, A: 2 articles (06/2001 - 12/2000)
10.	Fan, Yajuan: 1 article (04/2022)

Related Diseases

1.	Gingivitis 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. " 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. "
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 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." 08/01/2008 - "Identification of two novel beta-mannosidosis-associated sequence variants: biochemical analysis of beta-mannosidase (MANBA) missense mutations."
3.	Mitochondrial Diseases (Mitochondrial Disease) 04/01/2022 - "The study findings support the idea that the MAPK/ERK signaling pathway and mitochondrial dysfunction are involved in the pathogenesis of ASD, while a deficiency in beta-mannosidase might play a role in the development of ADHD. "
4.	Attention Deficit Disorder with Hyperactivity (Attention Deficit Hyperactivity Disorder) 04/01/2022 - "The study findings support the idea that the MAPK/ERK signaling pathway and mitochondrial dysfunction are involved in the pathogenesis of ASD, while a deficiency in beta-mannosidase might play a role in the development of ADHD. "
5.	Lysosomal Storage Diseases (Lysosomal Storage Disease) 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." 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. "

Related Drugs and Biologics

1.	alpha-Mannosidase (LAMAN)
2.	Hexosaminidase A (Hex A)
3.	Enzymes
4.	Oligosaccharides
5.	Glycoproteins (Glycoprotein)
6.	beta-Galactosidase (Lactaid)
7.	Glycoside Hydrolases (Endoglycosidases)
8.	Glucuronidase
9.	alpha-L-Fucosidase (Fucosidase)
10.	beta-N-Acetylhexosaminidases

Related Therapies and Procedures

1.	Bone Marrow Transplantation (Transplantation, Bone Marrow)