Recombinant GBA anticorps

N° du produit ABIN7828226

L’anticorps anti-GBA Monoclonal Lapin est utilisé pour la détection de GBA dans des échantillons de Humain, Souris et Rat. Il a été validé pour WB et IHC.

Aperçu rapide pour Recombinant GBA anticorps (ABIN7828226)

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

Type d'anticorp: Recombinant Antibody

Reactivité: Humain, Souris, Rat

Hôte: Lapin

Clonalité: Monoclonal

Conjugué: Cet anticorp GBA est non-conjugé

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

Clone: A678

Détail du produit anti-GBA anticorps

Fonction: Recombinant GBA Monoclonal Antibody

Purification: Protein A purified

Immunogène: Recombinant human GBA fragment

Isotype: IgG, kappa

Information d'application

Indications d'application: WB 1:1000-1:20000,IHC 1:50

Restrictions: For Research Use only

Stockage

Concentration: 1 mg/mL

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

Agent conservateur: ProClin

Précaution d'utilisation: This product contains ProClin: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.

Stock: -20 °C

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

Date de péremption: 12 months

Détails sur GBA

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

Autre désignation: GBA

Sujet: 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 [

Poids moléculaire:

Calculated MW: 60 kDa

Observed MW: 60 kDa

UniProt: P04062

Pathways: Cellular Glucan Metabolic Process