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Lysosomal alpha-mannosidase
- Molecular classification
- Enzyme, Glycoside hydrolase family GH38
01
Overview
Lysosomal alpha-mannosidase (LAMAN) is an acidic exoglycosidase enzyme essential for the catabolism of N-linked carbohydrates released during glycoprotein turnover. It cleaves α-1,2, α-1,3, and α-1,6 mannosidic linkages in oligosaccharides within lysosomes. Deficiency or dysfunction of LAMAN leads to alpha-mannosidosis, a rare autosomal recessive lysosomal storage disorder characterized by accumulation of undegraded oligosaccharides within cells. Mutations in MAN2B1 are responsible for this disease.
- Other names
- Alpha-mannosidase B deficiencyLysosomal alpha-D-mannosidase deficiencyMannosidosisMannosidosis, alpha B
02
Biological functions
Glycoprotein catabolismOligosaccharide degradationLysosomal protein turnover
03
Disease associations
Alpha-mannosidosisLysosomal storage disorder
04
Safety considerations
Progressive neurological decline (in alpha-mannosidosis)Immune deficiency (in alpha-mannosidosis)
05
Biomarkers
Undegraded oligosaccharides (in alpha-mannosidosis)
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