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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.
Also Known As:
Lysosomal alpha-D-Mannosidase Deficiency; 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; Mannosidosis, alpha B, Lysosomal
Networked: 107 relevant articles (9 outcomes, 14 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Lysosomal Storage Diseases (Lysosomal Storage Disease)
2. Mucolipidoses (Sialidosis)
3. alpha-Mannosidosis
4. Mucopolysaccharidoses
5. Fucosidosis

Experts

1. Fogh, Jens: 5 articles (01/2015 - 09/2004)
2. Saftig, Paul: 5 articles (01/2013 - 09/2004)
3. Hopwood, J J: 5 articles (01/2008 - 05/2001)
4. Malm, Dag: 4 articles (01/2013 - 04/2004)
5. Hopwood, John J: 4 articles (09/2006 - 03/2002)
6. Nilssen, Øivind: 3 articles (01/2015 - 07/2004)
7. Dali, Christine I: 3 articles (01/2015 - 12/2011)
8. Lund, Allan Meldgaard: 3 articles (01/2015 - 12/2011)
9. Vite, Charles H: 3 articles (01/2010 - 03/2005)
10. Andersson, Claes: 3 articles (01/2010 - 09/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to alpha-Mannosidosis:
1. alpha-Mannosidase (LAMAN)IBA
2. Carbon MonoxideIBA
3. SwainsonineIBA
4. OligosaccharidesIBA
5. LectinsIBA
6. ParaffinIBA
7. Peroxidase (Myeloperoxidase)IBA
8. G(M2) Ganglioside (Ganglioside GM2)IBA
9. Formaldehyde (Formol)FDA Link
10. Biotin (Vitamin H)FDA Link

Therapies and Procedures

1. Enzyme Replacement Therapy
2. Bone Marrow Transplantation (Transplantation, Bone Marrow)
3. Injections
4. Hematopoietic Stem Cell Transplantation
5. Transplants (Transplant)