Preferred Label : alpha-Mannosidase;

MeSH definition : An enzyme that catalyzes the HYDROLYSIS of terminal, non-reducing alpha-D-mannose residues in alpha-D-mannosides. The enzyme plays a role in the processing of newly formed N-glycans and in degradation of mature GLYCOPROTEINS. There are multiple isoforms of alpha-mannosidase, each having its own specific cellular location and pH optimum. Defects in the lysosomal form of the enzyme results in a buildup of mannoside intermediate metabolites and the disease ALPHA-MANNOSIDOSIS.;

MeSH synonym : mannohydrolase, alpha-d-mannoside; alpha d mannosidase; alpha-d-mannoside mannohydrolase; alpha d mannoside mannohydrolase; neutral alpha-mannosidase; neutral alpha mannosidase; alpha-mannosidase, neutral; alpha-d-mannosidase; alpha mannosidase;

MeSH hyponym : lysosomal alpha-Mannosidase; Lysosomal alpha Mannosidase; alpha-Mannosidase, Lysosomal; LAMAN; alpha Mannosidase B; Mannosidase B, alpha;

MeSH Related Number : lysosomal alpha-Mannosidase; EC 3.2.1.24;

Registry Number MeSH : EC 3.2.1.24;

Related CAS MeSH : EC 3.2.1.24 (Lysosomal alpha-Mannosidase);

MeSH annotation : /DEFIC: consider also ALPHA-MANNOSIDOSIS;

Is substance : O;

UNII : EC 3.2.1.24;

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An enzyme that catalyzes the HYDROLYSIS of terminal, non-reducing alpha-D-mannose residues in alpha-D-mannosides. The enzyme plays a role in the processing of newly formed N-glycans and in degradation of mature GLYCOPROTEINS. There are multiple isoforms of alpha-mannosidase, each having its own specific cellular location and pH optimum. Defects in the lysosomal form of the enzyme results in a buildup of mannoside intermediate metabolites and the disease ALPHA-MANNOSIDOSIS.

https://www.has-sante.fr/portail/jcms/c_2894917/en/lamzede
https://www.has-sante.fr/portail/jcms/c_2894917/fr/lamzede-velmanase-alpha-enzyme
2018
false
false
false
France
alpha-Mannosidase
recombinant proteins
infusions, intravenous
alpha-Mannosidosis
enzyme replacement therapy
velmanase alfa
orphan drug production
treatment outcome
guidelines for drug use
evaluation of the transparency committee

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https://www.ema.europa.eu/medicines/human/EPAR/Lamzede
2018
false
false
false
United Kingdom
French
English
treatment outcome
syndication feed
summary of product characteristics
package leaflet
drug evaluation
drug approval
europe
alpha-Mannosidosis
enzyme replacement therapy
velmanase alfa
alpha-Mannosidase
alpha-Mannosidase
Velmanase Alfa
Velmanase Alfa
adult
child
aged
infusions, intravenous
pregnancy
breast feeding
recombinant proteins
alpha-Mannosidase

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28/04/2025


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