化驗
≥95% (HPLC)
形狀
solid
顏色
white
溶解度
DMSO: 1 mg/mL
一般說明
A cell-permeable bisnaphthalimide compound that acts as a substrate-competitive and selective inhibitor against human β-N-acetyl hexosaminidase (IC50 = 6.0 and 3.1 μM for hHex A and hHex B, respectively), while exhibiting much reduced potency against Jack Bean or bacterial Hex (IC50 = 280 & >500 μM for JBHex & SpHex, respectively) and little or no activity against SmHex or hOGN. Shown to function as a pharmacological chaperone and effectively enhance the transport of mutant Hex alpha subunit from the ER to lysosome in cells derived from ISD and ATSD patients.
The β-Hexosaminidase Inhibitor controls the biological activity of β-Hexosaminidase. This small molecule/inhibitor is primarily used for Protease Inhibitors applications.
重構
Following reconstitution, aliquot and freeze (-20°C). Stock solutions are stable for up to 3 months at 20°C.
儲存類別代碼
10-13 - German Storage Class 10 to 13
Chemistry & biology, 14(2), 153-164 (2007-02-24)
The adult forms of Tay-Sachs and Sandhoff diseases result when the activity of beta-hexosaminidase A (Hex) falls below approximately 10% of normal due to decreased transport of the destabilized mutant enzyme to the lysosome. Carbohydrate-based competitive inhibitors of Hex act
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