Alpha-Mannosidosis is a lysosomal storage disorder caused by deficient activity of the enzyme alpha-D-mannosidase (EC 3.2.1.24). In humans it is known to be caused by an autosomal recessive genetic mutation. causes sugar build up and impairs cell function. Complete absence of functional enzyme leads to death during early childhood due to deterioration of the central nervous system. Enzyme with low residual activity leads to a milder type of the disease, with symptoms like reduced hearing, mental retardation, susceptibility to bacterial infections and skeletal deformities. Miglitol (Axon 2067), an alpha-glucosidase (EC 3.2.1.20) inhibitor, is an oral antihyperglycaemic agent is indicated for the treatment of patients with type 2 diabetes mellitus.