CST, 88162S, GCase/GBA (E2R1L) Rabbit Monoclonal Antibody

Monoclonal Antibody for studying GBA mouse. Validated for Western Blotting,Simple Western™. Available in 2 sizes. Highly specific and rigorously validated in-house, GCase/GBA (E2R1L) Rabbit Monoclonal Antibody (CST #88162) is ready to ship. Product Usage Information Western Blotting: 1:1000 Simple Western™: 1:10 - 1:50 Storage Supplied in 10 mM sodium HEPES (pH 7.5), 150 mM NaCl, 100 µg/mL BSA, 50% glycerol, and less than 0.02% sodium azide. Store at -20°C. Do not aliquot the antibody. For a carrier free (BSA and azide free) version of this product see product # 80765 . Protocol Available protocols: Western Blotting Specificity / Sensitivity GCase/GBA (E2R1L) Rabbit Monoclonal Antibody recognizes endogenous levels of total GCase/GBA protein. Species Reactivity: Human, Mouse, Rat Source / Purification Monoclonal antibody is produced by immunizing animals with recombinant protein specific to the amino terminus of mouse GCase/GBA protein. Background β-glucocerebrosidase (GCase) is a lysosomal enzyme that catalyzes the hydrolysis of glucocerebroside into free ceramide and glucose (1). Lysosomal breakdown of glucocerebroside is required for cellular metabolism of complex lipids and proper turnover of cellular membrane (2). In the absence of , the gene that encodes GCase, autophagic lysosome reformation is altered, suggesting that GCase activity is critical to maintain functional lysosomes. The cellular function of lysosomes is to degrade and recycle cellular waste to maintain proper cellular energy metabolism. Mutations in human cause deficiency in GCase, resulting in the accumulation of lysosomal glucocerebroside. Macrophages are particularly sensitive to lysosomal glucocerebroside accumulation due to their role in phagocytosis-mediated breakdown of cellular debris and dying cells. Gaucher disease, a rare autosomal recessive lysosomal storage disorder that is genetically linked to , is marked by engorged "Gaucher cell" macrophages in the spleen, liver, and bone marrow (3). Gaucher disease patients also exhibit neurological manifestations. mutations are the most common genetic risk factor for Parkinson's disease (PD), a neurodegenerative disease characterized by the loss of dopaminergic neurons in the substantia nigra with formation of α-synuclein-rich Lewy bodies in surviving neurons (4,5). mutations may play a direct role in accumulation of α-synuclein by mechanisms that are poorly understood, but may include mislocalization of lysosomal GCase causing impaired lysosomal degradation of α-synuclein (6,7). Alternate Names acid beta glucosidase; Acid beta-glucosidase; Beta-glucocerebrosidase; betaG; betaGC; Cholesterol glucosyltransferase; Cholesteryl-beta-glucosidase; D-glucosyl-N-acylsphingosine glucohydrolase; Gba; GBA1; GC; GCa; GCase; GLCM; GLUC; glucocerebrosidase; glucosidase, beta, acid; Glucosylceramidase; Lysosomal acid GCase; Lysosomal acid glucosylceramidase; SGTase Specification REACTIVITY: H M R SENSITIVITY: Endogenous MW (kDa): 65 Source/Isotype: Rabbit IgG