Glucosylceramidase/GBA Antibody (812201) [Unconjugated]

This product is produced by and ships from R&D Systems, Inc., a Bio-Techne brand.

Glucosylceramidase is a lysosomal enzyme that cleaves the beta-glucosidic linkage of glucosylceramide (1, 2), an intermediate in glycolipid metabolism. The mature enzyme has 497 amino acids with a molecular weight of 62 kDa (3). Glycosylation occurs at four of five N-glycosylation sites and is essential for the trafficking and activity of the enzyme (4). The enzyme is activated in lysosomes by saposin C, although the mechanism of activation is not well understood (5). Defects in Glucosylceramidase are the cause of Gaucher disease, also known as glucocerebrosidase deficiency (6). Gaucher disease is the most prevalent lysosomal storage disease, characterized by accumulation of glucosylceramide in the reticulo-endothelial system. Symptoms of Gaucher disease may include enlarged spleen and liver, liver malfunction, skeletal disorders and bone lesions, severe neurologic complications, swelling of lymph nodes, anemia, low blood platelets and yellow fatty deposits on the white of the eye (7). Currently, enzyme replacement therapy is used to treat patients with the disease (8, 9).

This product is for research use only and is not approved for use in humans or in clinical diagnosis. Primary Antibodies are guaranteed for 1 year from date of receipt.

We have publications tested in 1 confirmed species: Human.

We have publications tested in 2 applications: IHC, Western Blot.

Showing Publications 1 - 7 of 7.

Publications using MAB7410	Applications	Species
Jong, T;Gehrlein, A;Sidransky, E;Jagasia, R;Chen, Y; Characterization of Novel Human ?-glucocerebrosidase Antibodies for Parkinson Disease Research bioRxiv : the preprint server for biology 2023-09-15 [PMID: 37886493] (Western Blot)	Western Blot
SE Davis, AK Cook, JA Hall, Y Voskobiyny, NV Carullo, NR Boyle, AR Hakim, KM Anderson, KP Hobdy, DA Pugh, CF Murchison, LJ McMeekin, M Simmons, KA Margolies, RM Cowell, AL Nana, S Spina, LT Grinberg, BL Miller, WW Seeley, AE Arrant Patients with sporadic FTLD exhibit similar increases in lysosomal proteins and storage material as patients with FTD due to GRN mutations Acta neuropathologica communications, 2023-04-28;11(1):70. 2023-04-28 [PMID: 37118844] (IHC, Human)	IHC	Human
Oliver B. Davis, Hijai R. Shin, Chun-Yan Lim, Emma Y. Wu, Matthew Kukurugya, Claire F. Maher et al. NPC1-mTORC1 Signaling Couples Cholesterol Sensing to Organelle Homeostasis and Is a Targetable Pathway in Niemann-Pick Type C Developmental Cell 2/1/2021 [PMID: 33308480]
Andrew E. Arrant, Jonathan R. Roth, Nicholas R. Boyle, Shreya N. Kashyap, Madelyn Q. Hoffmann, Charles F. Murchison et al. Impaired beta -glucocerebrosidase activity and processing in frontotemporal dementia due to progranulin mutations Acta Neuropathologica Communications 12/23/2019 [PMID: 31870439]
Yoon-Myung Kim, Mi-Sun Yum, Sun Hee Heo, Taeho Kim, Hee Kyung Jin, Jae-sung Bae et al. Pharmacologic properties of high-dose ambroxol in four patients with Gaucher disease and myoclonic epilepsy Journal of Medical Genetics 2/1/2020 [PMID: 31649052]
Jessie Adams, Melissa Feuerborn, Joshua A. Molina, Alexa R. Wilden, Babita Adhikari, Theodore Budden et al. Autophagy–lysosome pathway alterations and alpha-synuclein up-regulation in the subtype of neuronal ceroid lipofuscinosis, CLN5 disease Scientific Reports 1/17/2019 [PMID: 30655561]
Atashrazm F, Hammond D, Perera G et al. Reduced glucocerebrosidase activity in monocytes from patients with Parkinson's disease. Sci Rep. 10/18/2018 [PMID: 30337601]

Array MAB7410

• Glucosylceramidase/GBA Antibodies
• Glucosylceramidase/GBA ELISA Kits
• Glucosylceramidase/GBA Kit
• Glucosylceramidase/GBA Lysates
• Glucosylceramidase/GBA Protein
• Glucosylceramidase/GBA Proteins and Enzyme