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
1730 (+)-Kifunensine α-mannosidase inhibitor €120.00
3181 BI 99179 Potent, selective and orally active inhibitor of type I fatty acid synthase (FAS) €150.00
3182 BI 99990 Negative control of BI 99179 as a selective inhibitor of type I fatty acid synthase €130.00
3570 CD38 inhibitor compound 78c Potent, specific, reversible, and uncompetitive inhibitor of CD38 €140.00
3517 DSRM-3716 Potent and selective SARM1 NADase inhibitor €70.00
2617 GSK837149 Selective inhibitor of human fatty acid synthase (FAS) €120.00
2067 Miglitol α-glucosidase inhibitor; oral anti-diabetic €55.00
3136 Oseltamivir phosphate Selective and orally available inhibitor of influenza virus neuraminidases €50.00
2934 TH 5487 Potent and selective active-site OGG1 inhibitor €125.00

9 Item(s)

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