GBA (1P9) Rabbit Monoclonal Antibody
Konjugasyon: Unconjugated
Recombinant rabbit monoclonal antibody
Uygulama
Reaktivite
Human
Gen Adı
GBA
Saklama
Aliquot and store at -20°C (valid for 12 months). Avoid freeze/thaw cycles.
Özet
| Ürün Adı | GBA (1P9) Rabbit Monoclonal Antibody |
| Açıklama | Recombinant rabbit monoclonal antibody |
| Konak | Rabbit |
| Reaktivite | Human |
| Konjugasyon | Unconjugated |
| Modifikasyon | Unmodified |
| İzotip | IgG |
| Klonalite | Monoclonal |
| Form | Liquid |
| Konsantrasyon | Unconjugated |
| Saklama | Aliquot and store at -20°C (valid for 12 months). Avoid freeze/thaw cycles. |
| Nakliye | Ice bags. |
| Tampon | Rabbit IgG in phosphate buffered saline , pH 7.4, 150mM NaCl, 0.02% New type preservative N and 50% glycerol. Store at +4°C short term. Store at -20°C long term. Avoid freeze / thaw cycle. |
| Saflaştırma | Affinity purification |
Antijen Bilgisi
| Gen Adı | GBA |
| Alternatif İsimler | Alglucerase; betaGC; GBA1; GCase; GCB; GLUC; Glucosylceramidase; Imiglucerase; |
| Gen Kimliği | 2629 |
| SwissProt Kimliği | P04062 |
| İmmünojen | A synthetic peptide of human GBA |
Uygulama
| Uygulama | WB,IHC |
| Seyreltme Oranı | WB 1:1000-1:5000,IHC 1:50-1:100 |
| Moleküler Ağırlık | 60kDa |
Araştırma Alanı
Arka Plan
| Defects in GBA are the cause of Gaucher disease (GD) [MIM:230800]; also known as glucocerebrosidase deficiency. GD is the most prevalent lysosomal storage disease, characterized by accumulation of glucosylceramide in the reticulo-endothelial system. Glucosylceramidase that catalyzes, within the lysosomal compartment, the hydrolysis of glucosylceramide/GlcCer into free ceramide and glucose (PubMed:9201993, PubMed:24211208, PubMed:15916907). Thereby, plays a central role in the degradation of complex lipids and the turnover of cellular membranes (PubMed:27378698). Through the production of ceramides, participates in the PKC-activated salvage pathway of ceramide formation (PubMed:19279011). Also plays a role in cholesterol metabolism (PubMed:24211208, PubMed:26724485). May either catalyze the glucosylation of cholesterol, through a transglucosylation reaction that transfers glucose from glucosylceramide to cholesterol (PubMed:24211208, PubMed:26724485). The short chain saturated C8:0- GlcCer and the mono-unsaturated C18:0-GlcCer being the most effective glucose donors for that transglucosylation reaction (PubMed:24211208). Under specific conditions, may alternatively catalyze the reverse reaction, transferring glucose from cholesteryl-beta-D-glucoside to ceramide (PubMed:26724485). Finally, may also hydrolyze cholesteryl- beta-D-glucoside to produce D-glucose and cholesterol (PubMed:24211208, PubMed:26724485). |
