Molecular Formula | C6H14ClNO4 |
Molar Mass | 199.63 |
Melting Point | 260 |
Solubility | Methanol (Slightly), Water (Slightly) |
Appearance | White crystalline solid |
Color | White to Brown |
Storage Condition | 2-8°C |
In vitro study | Both IC 50 and K i values of Migalastat (GR181413A) hydrochloride toward human lysosomal a-Gal A are 0.04 μM. |
In vivo study | Fabry disease is an X-linked recessive disorder caused by the deficient activity of α-galactosidase A. α-Gal A activity in heart, kidney, spleen, and liver is increased dose- and time-dependently in transgenic mice that express human mutant alpha-Gal A with Migalastat (GR181413A) hydrochloride treatment. The half-life of DGJ is less than 1 day in all major issues and that of the enzyme synthesized during the DGJ treatment period is approximately 4 days. Oral administration of Migalastat (GR181413A) hydrochloride reduces tissue GL-3 in fabry transgenic mice, and in urine and kidneys of some FD patients. Oral administration of Migalastat (GR181413A) hydrochloride to transgenic mice reduces elevated lyso-Gb3 levels up to 64%, 59%, and 81% in kidney, heart, and skin, respectively, generally equal to or greater than observed for GL-3. |
Hazard Symbols | Xi - Irritant |
Risk Codes | 36/37/38 - Irritating to eyes, respiratory system and skin. |
Safety Description | S26 - In case of contact with eyes, rinse immediately with plenty of water and seek medical advice. S36 - Wear suitable protective clothing. |
WGK Germany | 3 |
HS Code | 29333990 |
Hazard Note | Irritant |
1mg | 5mg | 10mg | |
---|---|---|---|
1 mM | 5.009 ml | 25.046 ml | 50.093 ml |
5 mM | 1.002 ml | 5.009 ml | 10.019 ml |
10 mM | 0.501 ml | 2.505 ml | 5.009 ml |
5 mM | 0.1 ml | 0.501 ml | 1.002 ml |