Diseases

alpha-Mannosidosis

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.

National Library of MedicineMedical Subject Headings2026

Structured Summary

Abstract

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.

MeSH Record

Classification

Broader headings

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MeSH Record

See Also

MeSH Record

Synonyms

21 entry terms
  • 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
  • Deficiencies, Lysosomal alpha-D-Mannosidase
  • Deficiencies, alpha-Mannosidase
  • Deficiency, Lysosomal alpha-D-Mannosidase
  • Deficiency, alpha-Mannosidase
  • Lysosomal alpha D Mannosidase Deficiency
  • Lysosomal alpha-D-Mannosidase Deficiencies
  • alpha Mannosidase Deficiency
  • alpha Mannosidosis
  • alpha-D-Mannosidase Deficiencies, Lysosomal
  • alpha-D-Mannosidase Deficiency, Lysosomal
  • alpha-Mannosidase Deficiencies
  • alpha-Mannosidoses

MeSH Record

Aspects Covered

34 allowable subheadings

Indexed with the subheadings blood, cerebrospinal fluid, chemically induced, classification, complications, diagnosis, diagnostic imaging, diet therapy, drug therapy, economics, embryology, enzymology, epidemiology, ethnology, etiology, genetics, history, immunology, metabolism, microbiology, mortality, nursing, parasitology, pathology, physiopathology, prevention & control, psychology, radiotherapy, rehabilitation, surgery, therapy, urine, veterinary, virology.

MeSH Record

History Note

2004(1985)

MeSH Record

Previous Indexing

  • Carbohydrate Metabolism, Inborn Errors (1966-1984)
  • Mannose (1966-1984)
  • Mannosidases (1975-1984)
  • Mannosidosis (1985-2003)
  • Metabolism, Inborn Errors (1966-1984)

MeSH Hierarchy

Tree Numbers

  1. C16w.320w.565w.202w.607w.500w
  2. C16w.320w.565w.595w.577w.500w
  3. C18.452w.648w.202w.607w.500w
  4. C18.452w.648w.595w.577w.500w

AMA Style

References

  1. National Library of Medicine. alpha-Mannosidosis. Medical Subject Headings (MeSH). 2026. Unique ID D008363. http://id.nlm.nih.gov/mesh/2026/D008363
  2. alpha-Mannosidosis. In: Wikipedia. https://en.wikipedia.org/wiki/Alpha-mannosidosis
  3. alpha-Mannosidosis. In: Wikidata. https://www.wikidata.org/wiki/Q250449