Please refer to the specific buffer information in the hardcopy of datasheet or the lot-specific COA.
产品使用信息
应用方法
稀释比例
ELISA
1:2000-1:20000
稳定性和存储
Use a manual defrost freezer and avoid repeated freeze thaw cycles. Store at 2 to 8°C for frequent use. Store at -20 to -80°C for twelve months from the date of receipt.
背景
Lysosomal acid glucosylceramidase (GBA) is a ~59 kDa protein. Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramides/GlcCers (such as beta-D-glucosyl-(1<->1')-N-acylsphing-4-enine) into free ceramides (such as N-acylsphing-4-enine) and glucose. Plays a central role in the degradation of complex lipids and the turnover of cellular membranes. Through the production of ceramides, participates in the PKC-activated salvage pathway of ceramide formation. Catalyzes the glucosylation of cholesterol, through a transglucosylation reaction where glucose is transferred from GlcCer to cholesterol. GlcCer containing mono-unsaturated fatty acids (such as beta-D-glucosyl-N-(9Z-octadecenoyl)-sphing-4-enine) are preferred as glucose donors for cholesterol glucosylation when compared with GlcCer containing same chain length of saturated fatty acids (such as beta-D-glucosyl-N-octadecanoyl-sphing-4-enine).
1. Ron, I. et al. (2005) Blood cells, molecules & diseases 35, 57-65. PMID: 15916907
2. Akiyama, H. et al. (2013) Biochemical and biophysical research communications 441, 838-43. PMID: 24211208
3. Akiyama, H. et al. (2020) The Journal of biological chemistry 295, 5257-5277. PMID: 32144204
4. Bannink, S. et al. (2024) Journal of lipid research 65, 100670. PMID: 39395789
5. Vaccaro, AM. et al. (1997) The Journal of biological chemistry 272, 16862-7. PMID: 9201993
6. Magalhaes, J. et al. (2016) Human molecular genetics 25, 3432-3445. PMID: 27378698
7. Kitatani, K. et al. (2009) The Journal of biological chemistry 284, 12972-8. PMID: 19279011
8. Marques, AR. et al. (2016) Journal of lipid research 57, 451-63. PMID: 26724485
9. Boer, DE. et al. (2021) Journal of lipid research 62, 100018. PMID: 33361282
