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Alpha-Mannosidosis
Condition Overview: Alpha-mannosidosis is one of a group of very rare inherited disorders known as glycoprotein and related storage diseases. These disorders are caused by a defect in the breakdown of complex molecules in the cells, as a result of an enzyme deficiency. The enzyme that is lacking is known as alpha-D-mannosidase. The molecules that are not broken down are stored in the small components within cells known as lysosomes. The approximately 50 diseases in which this occurs are known as lysosomal storage diseases. In addition to alpha mannosidosis and a related disorder, beta mannosidosis, the lysosomal storage disorders include Fabry disease, Gaucher disease, cystinosis, aspartylglycosaminuria, the mucopolysaccharidoses, the mucolipidoses, and others.There are two types of alpha-mannosidosis: type I and type II. Type I is much less common. Its symptoms may begin within the first year of life. Type II, the milder form, may begin during the juvenile or adult years. |
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