anti-gba rabbit mab

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Recombinant Rabbit Monoclonal Anti-GBA Rabbit mAb Clone Number: JRMR-10681(2)-6 Host: Rabbit Clonality: Recombinant Monoclonal Applications： WB IHC-P Reactivity: Human, Mouse, Rat Synonyms: GCase, Beta-GC, Acid beta-glucosidase, SGTase Product Size 100 μl ADD TO CART BUY NOW Quantity Shipping: Ambient temperature Order online or send purchase order to [email protected] FAQ Technical Support Protocols General Information Product Usage Information Properties Target Information Images Recommended Products References BUY NOW General Information Isotype IgG Conjugate Unconjugated Synonyms GCase, Beta-GC, Acid beta-glucosidase, SGTase UniProt ID P04062 Immunogen MW (kDa) Specificity Product Usage Information Applications Dilution Recommended Species WB 1:1000 – 1:20000 Human, Mouse, Rat IHC-P 1:50 Human Properties Purity Protein A purified Constituents PBS, Glycerol, BSA Storage Store at -20°C. Avoid freeze/thaw cycles. Stability Stable for 12 months from date of receipt/reconstitution. Target Information Background Beta-Glucosylceramidase (β-GC) is a lysosomal enzyme that catalyzes the hydrolysis of glucocerebroside into free ceramide and glucose. Lysosomal breakdown of glucocerebroside is required for cellular metabolism of complex lipids and proper turnover of cellular membrane. In the absence of GBA, the gene that encodes β-GC, autophagic lysosome reformation is altered, suggesting that β-GC 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 GBA cause deficiency in β-GC, 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 GBA, is marked by engorged “Gaucher cell” macrophages in the spleen, liver, and bone marrow. GBA 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. GBA mutations may play a direct role in accumulation of α-synuclein by mechanisms that are poorly understood, but may include mislocalization of lysosomal β-GC causing impaired lysosomal degradation of α-synuclein. Cellular location Lysosome Images WB Blocking buffer: 5% NFDM/TBSTPrimary Ab dilution: 1:20000Primary Ab incubation condition: 2 hours at room temperatureSecondary Ab: Goat Anti-Rabbit IgG H&L pAb (HRP Conjugate)Lysate: 1: MCF-7, 2: Saos-2, 3: SH-SY5Y, 4: 4T1, 5: C6Protein loading amount: 20 μgExposure time: 60 sPredicted band size: 60 kDaObserved band size: 60 kDa IHC-P Tissue: Human kidneySection type: Formalin-fixed & paraffinembedded sectionRetrieval method: High temperature and high pressureRetrieval buffer: Tris/EDTA buffer, pH 9.0Primary Ab dilution: 1:50Primary Ab incubation condition: 1 hour at room temperatureSecondary Ab: Anti-Rabbit and Mouse Polymer HRP (Ready to Use)Counter stain: Hematoxylin (Blue)Description: The brown color represents the positive signal observed with :
Recombinant Rabbit Monoclonal Anti-GBA Rabbit mAb Clone Number: JRMR-10681(2)-6 Host: Rabbit Clonality: Recombinant Monoclonal Applications： WB IHC-P Reactivity: Human, Mouse, Rat Synonyms: GCase, Beta-GC, Acid beta-glucosidase, SGTase Product Size 100 μl ADD TO CART BUY NOW Quantity Shipping: Ambient temperature Order online or send purchase order to [email protected] FAQ Technical Support Protocols General Information Product Usage Information Properties Target Information Images Recommended Products References BUY NOW General Information Isotype IgG Conjugate Unconjugated Synonyms GCase, Beta-GC, Acid beta-glucosidase, SGTase UniProt ID P04062 Immunogen MW (kDa) Specificity Product Usage Information Applications Dilution Recommended Species WB 1:1000 – 1:20000 Human, Mouse, Rat IHC-P 1:50 Human Properties Purity Protein A purified Constituents PBS, Glycerol, BSA Storage Store at -20°C. Avoid freeze/thaw cycles. Stability Stable for 12 months from date of receipt/reconstitution. Target Information Background Beta-Glucosylceramidase (β-GC) is a lysosomal enzyme that catalyzes the hydrolysis of glucocerebroside into free ceramide and glucose. Lysosomal breakdown of glucocerebroside is required for cellular metabolism of complex lipids and proper turnover of cellular membrane. In the absence of GBA, the gene that encodes β-GC, autophagic lysosome reformation is altered, suggesting that β-GC 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 GBA cause deficiency in β-GC, 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 GBA, is marked by engorged “Gaucher cell” macrophages in the spleen, liver, and bone marrow. GBA 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. GBA mutations may play a direct role in accumulation of α-synuclein by mechanisms that are poorly understood, but may include mislocalization of lysosomal β-GC causing impaired lysosomal degradation of α-synuclein. Cellular location Lysosome Images WB Blocking buffer: 5% NFDM/TBSTPrimary Ab dilution: 1:20000Primary Ab incubation condition: 2 hours at room temperatureSecondary Ab: Goat Anti-Rabbit IgG H&L pAb (HRP Conjugate)Lysate: 1: MCF-7, 2: Saos-2, 3: SH-SY5Y, 4: 4T1, 5: C6Protein loading amount: 20 μgExposure time: 60 sPredicted band size: 60 kDaObserved band size: 60 kDa IHC-P Tissue: Human kidneySection type: Formalin-fixed & paraffinembedded sectionRetrieval method: High temperature and high pressureRetrieval buffer: Tris/EDTA buffer, pH 9.0Primary Ab dilution: 1:50Primary Ab incubation condition: 1 hour at room temperatureSecondary Ab: Anti-Rabbit and Mouse Polymer HRP (Ready to Use)Counter stain: Hematoxylin (Blue)Description: The brown color represents the positive signal observed with