Recombinant GBA antibody

Catalog No. ABIN7828226

The Rabbit Monoclonal anti-GBA antibody is suitable to detect GBA in samples from Human, Mouse and Rat. It has been validated for WB and IHC.

Quick Overview for Recombinant GBA antibody (ABIN7828226)

Target: GBA (Glucosidase, Beta, Acid (GBA))

Antibody Type: Recombinant Antibody

Reactivity: Human, Mouse, Rat

Host: Rabbit

Clonality: Monoclonal

Conjugate: This GBA antibody is un-conjugated

Application: Western Blotting (WB), Immunohistochemistry (IHC)

Clone: A678

Product Details anti-GBA Antibody

Purpose: Recombinant GBA Monoclonal Antibody

Purification: Protein A purified

Immunogen: Recombinant human GBA fragment

Isotype: IgG, kappa

Application Details

Application Notes: WB 1:1000-1:20000,IHC 1:50

Restrictions: For Research Use only

Handling

Concentration: 1 mg/mL

Buffer: PBS, 50 % glycerol, 0.05 % Proclin 300, 0.05 % protein protectant.

Preservative: ProClin

Precaution of Use: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Storage: -20 °C

Storage Comment: Store at -20°C Valid for 12 months. Avoid freeze / thaw cycles.

Expiry Date: 12 months

Target Details for GBA

Target: GBA (Glucosidase, Beta, Acid (GBA))

Alternative Name: GBA

Background: GBA,GBA1,GCB,GLUC,GC,9-O-sialyl GD3,7-O-sialyl GD3,ACID,acid (includes glucosylceramidase),beta,Acid beta glucosidase,Acid beta-glucosidase,Alglucerase,Beta glucocerebrosidase,BETA GLUCOSIDASE,betaGC,Beta-glucocerebrosidase,D glucosyl N acylsphingosine glucohydrolase,D-glucosyl-N-acylsphingosine glucohydrolase,EC 3.2.1.45,Gba protein,GCase,GLCM,Glucocerebrosidase,Glucocerebrosidase (alt.),GLUCOCEREBROSIDASE PSEUDOGENE,Glucosidase,Glucosidase beta,Glucosylceramidase,Imiglucerase,Lysosomal glucocerebrosidase,OTTHUMP00000033992,OTTHUMP00000033993,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 ofGBA, 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 humanGBAcause 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 toGBA, is marked by engorged ""Gaucher cell"" macrophages in the spleen, liver, and bone marrow. GBAmutations 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.GBAmutations 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. Cat.No. Product Name Clone No. IF:{{item.impact}} Journal:{{item.journal}} ({{item.year}}) DOI:{{item.doi}} Reactivity:{{item.species}} Sample Type:{{item.sample_type}} Previous {{ page }} Next Q{{(FAQpage.currentPage - 1)*pageSize+index+1}}:{{item.name}} Previous {{ page }} Next [

Molecular Weight:

Calculated MW: 60 kDa

Observed MW: 60 kDa

UniProt: P04062

Pathways: Cellular Glucan Metabolic Process