Rekombinanter GBA Antikörper

Produktnummer ABIN7828226

Der Kaninchen Monoklonal anti-GBA Antikörper wird verwendet zum Nachweis von GBA in Proben von Human, Maus und Ratte. Er wurde validiert für WB und IHC.

Kurzübersicht für Rekombinanter GBA Antikörper (ABIN7828226)

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

Antikörpertyp: Recombinant Antibody

Reaktivität: Human, Maus, Ratte

Wirt: Kaninchen

Klonalität: Monoklonal

Konjugat: Dieser GBA Antikörper ist unkonjugiert

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

Klon: A678

Produktdetails anti-GBA Antikörper

Verwendungszweck: Recombinant GBA Monoclonal Antibody

Aufreinigung: Protein A purified

Immunogen: Recombinant human GBA fragment

Isotyp: IgG, kappa

Anwendungsinformationen

Applikationshinweise: WB 1:1000-1:20000,IHC 1:50

Beschränkungen: Nur für Forschungszwecke einsetzbar

Handhabung

Konzentration: 1 mg/mL

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

Konservierungsmittel: ProClin

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

Lagerung: -20 °C

Informationen zur Lagerung: Store at -20°C Valid for 12 months. Avoid freeze / thaw cycles.

Haltbarkeit: 12 months

Details zu GBA

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

Andere Bezeichnung: GBA

Hintergrund: 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 [

Molekulargewicht:

Calculated MW: 60 kDa

Observed MW: 60 kDa

UniProt: P04062

Pathways: Cellular Glucan Metabolic Process