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alpha-Mannosidosis MeSH Descriptor Data 2022


MeSH Heading
alpha-Mannosidosis
Tree Number(s)
C16.320.565.202.607.500
C16.320.565.595.577.500
C18.452.648.202.607.500
C18.452.648.595.577.500
Unique ID
D008363
RDF Unique Identifier
http://id.nlm.nih.gov/mesh/D008363
Scope Note
An inborn error of metabolism marked by a defect in the lysosomal isoform of ALPHA-MANNOSIDASE activity that results in lysosomal accumulation of mannose-rich intermediate metabolites. Virtually all patients have psychomotor retardation, facial coarsening, and some degree of dysostosis multiplex. It is thought to be an autosomal recessive disorder.
Entry Term(s)
Alpha-D-Mannosidosis
Alpha-Mannosidase B Deficiency
Alpha-Mannosidosis, Type I
Lysosomal Alpha B Mannosidosis
Lysosomal alpha-D-Mannosidase Deficiency
Mannosidosis, alpha B Lysosomal
Mannosidosis, alpha B, Lysosomal
alpha Mannosidase B Deficiency
alpha-Mannosidase Deficiency
Previous Indexing
Carbohydrate Metabolism, Inborn Errors (1966-1984)
Mannose (1966-1984)
Mannosidases (1975-1984)
Mannosidosis (1985-2003)
Metabolism, Inborn Errors (1966-1984)
See Also
alpha-Mannosidase
Public MeSH Note
2004; see MANNOSIDOSIS 1984-2003
History Note
2004(1985)
Date Established
1985/01/01
Date of Entry
1984/05/29
Revision Date
2013/07/08
alpha-Mannosidosis Preferred
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