Linkages alpha mannosidase. Medical search. Frequent questions

The enzyme catalyzes the hydrolysis of terminal, non-reducing alpha-D-mannose residues in alpha-D-mannosides, and can cleave all known types of alpha-mannosidic linkages. (wikipedia.org)
Chitinases are glycosyl hydrolases that catalyze the hydrolysis of the β-1,4 linkages in complex carbohydrates and those that contain GlcNAc. (researchgate.net)
Glycoside hydrolases that catalyze the hydrolysis of alpha or beta linked MANNOSE . (lookformedical.com)
Diseases caused by the loss of one or more enzymes involved in the hydrolysis of mannoside linkages (MANNOSIDASES). (ouhsc.edu)

Any member of the class of enzymes that catalyze the cleavage of the glycosidic linkage of glycosides and the addition of water to the resulting molecules. (bvsalud.org)

Final Report Summary - ALPHA-MAN (Clinical development of Enzyme Replacement Therapy in alpha-Mannosidosis patients using recombinant human enzyme. (europa.eu)
Alpha-Mannosidosis is caused by an enzyme defect due to mutations in the gene for lysosomal alpha-Mannosidase (LAMAN) affecting the lysosomal and cellular glycoprotein catabolism with severe consequences for the organism. (europa.eu)
Today, the most promising therapy for lysosomal storage disorders including alpha-Mannosidosis is Enzyme Replacement Therapy (ERT) where the respective enzyme lacking in the patient is produced by recombinant approaches and then introduced into the blood stream, from where it is internalized by the cells and reaches the lysosomes replacing the missing endogenous enzyme. (europa.eu)
Since pharmaceutical interest in this disease is low, two EU-supported projects (EURAMAN and HUE-MAN) within the 5th and 6th framework program, respectively have worked towards developing the recombinant human enzyme (rhLAMAN) as a therapeutic agent for patients suffering from alpha-Mannosidosis. (europa.eu)
The domain is predominantly found in the enzyme alpha-mannosidase. (wikipedia.org)
The defects in enzyme activity are primarily associated with genetic mutation of the genes that codes for a particular mannosidase isoenzyme. (ouhsc.edu)
An enzyme that hydrolyzes 1,6-alpha-glucosidic branch linkages in glycogen, amylopectin, and their beta-limit dextrins. (nih.gov)

This family includes Ruminococcus albus 4-O-beta-D-mannosyl-D-glucose phosphorylase (RaMP1) and beta-(1,4)-mannooligosaccharide phosphorylase (RaMP2), enzymes that phosphorolyze beta-mannosidic linkages at the non-reducing ends of their substrates, and have substantially diverse substrate specificity that are determined by three loop regions. (unl.edu)
Many GH43 enzymes display both alpha-L-arabinofuranosidase and beta-D-xylosidase activity using aryl-glycosides as substrates. (unl.edu)
This GH43_ HoAraf43-like subgroup includes enzymes that have been annotated as having xylan-digesting beta-xylosidase (EC 3.2.1.37) and xylanase (endo-alpha-L-arabinanase, EC 3.2.1.8) activities. (unl.edu)

Defects in the gene cause lysosomal alpha-mannosidosis (AM), a lysosomal storage disease characterised by the accumulation of unbranched oligo-saccharide chains. (wikipedia.org)

α(1-6) Core Mannosidase cleaves unbranched non-reducing terminal mannose, α(1-6) linked to the β-linked core mannose of the conserved mannosylchitobiose core of N-linked oligosaccharides. (ludger.com)
Amylo-α-1,6-glucosidase ( EC 3.2.1.33 ), or glucosidase , cleaves the remaining alpha-1,6 linkage, producing glucose and a linear chain of glycogen. (wikidoc.org)
[5] The mechanism by which the glucosidase cleaves the α -1,6-linkage is not fully known because the amino acids in the active site have not yet been identified. (wikidoc.org)

Phosphorylase can only cleave α-1,4- glycosidic bond between adjacent glucose molecules in glycogen but branches exist as α-1,6 linkages. (wikidoc.org)

The lysosomal storage disorder (LSD) alpha-Mannosidosis is a rare genetic disease and according to the EU regulations, designated as an "orphan" disease. (europa.eu)
Lysosomal alpha-mannosidase is necessary for the catabolism of N-linked carbohydrates released during glycoprotein turnover. (wikipedia.org)

To date, no specific treatment for alpha-Mannosidosis is available. (europa.eu)
The final goal of ALPHA-MAN was to make a future treatment for ALL alpha-Mannosidosis patients available and thereby dramatically improving their life expectancy and quality of life. (europa.eu)
In line with our plans we have been able to demonstrate the safety and clinical efficacy of rhLAMAN as an effective therapeutic agent for treatment of the human disease alpha-Mannosidosis in clinical trial Phases 1 and 2. (europa.eu)
In non-clinical studies we also found the optimal rhLAMAN dose for a maximal correction of visceral and central nervous system pathology following injection in immune-tolerant alpha Mannosidosis mice. (europa.eu)
Putative receptors that are responsible for rhLAMAN uptake into the brain across the Blood Brain Barrier were investigated and we were able to study the outcome of long-term ERT on the neuropathology and associated behavioural deficits observed in immune-tolerant alpha-Mannosidosis mice. (europa.eu)

Mutations in the alpha 1,2-mannosidase gene, MAN1B1, cause autosomal-recessive intellectual disability. (mpg.de)
In E. coli , Glucose transfer is performed by 4-alpha-glucanotransferase, a 78.5 kDa protein coded for by the gene malQ. (wikidoc.org)

it produces alpha-mannose 1-phosphate and glucose from 4-O-beta-D-mannosyl-D-glucose and inorganic phosphate, using a critical catalytic Asp as a proton donor. (unl.edu)

1,4 linkages in complex carbohydrates and those that contain GlcNAc. (researchgate.net)
Carbohydrates consisting of between two (DISACCHARIDES) and ten MONOSACCHARIDES connected by either an alpha- or beta-glycosidic link. (lookformedical.com)

To increase the capacity of studying genetic linkage or association, grandparents, parents, and siblings without lupus will also be recruited. (bioinf.org)

To create the large repertoire of glycan structures, each of these glycosyltransferases uses a single-nucleotide sugar substrate and forms specific linkages between one monosaccharide and a glycan precursor. (nature.com)

There is no detectable activity on α(2-8) or α(2-9) linkages or on branched α(2-3) or α(2-6) linkages. (ludger.com)

A domain, which is found in the central region adopts a structure consisting of three alpha helices, in an immunoglobulin/albumin-binding domain-like fold. (wikipedia.org)