Alpha-Mannosidosis is a lysosomal storage disorder caused by deficient activity of the enzyme alpha-D-mannosidase (EC 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[1]. Miglitol (Axon 2067), an alpha-glucosidase (EC inhibitor, is an oral antihyperglycaemic agent is indicated for the treatment of patients with type 2 diabetes mellitus[2].

[1] Adult alpha-mannosidosis: clinical progression in the absence of demyelination. A. Gutschalk,I. Harting, M. Cantz, C. Springer, K. Rohrschneider, H.M. Meinck. Neurology. 2004, 63, 1744-1746.
[2] Miglitol: a review of its therapeutic potential in type 2 diabetes mellitus. L.J. Scott, C.M. Spencer. Drugs. 2000, 9, 521-549.

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Axon ID Name Description From price
2617 GSK837149 Selective inhibitor of human fatty acid synthase (FAS) €120.00
1730 Kifunensine, (+)- α-mannosidase inhibitor €120.00
2067 Miglitol α-glucosidase inhibitor; oral anti-diabetic €55.00
2934 TH 5487 Potent and selective active-site OGG1 inhibitor €125.00

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