A4795
Niemann Pick C1 Rabbit monoclonal antibody
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- SKU:
- A4795
- Zusätzliche Namen:
- Niemann Pick C1|NPC|POGZ|SLC65A1
- Anwendung:
- ELISA, WB, IHC-P, IF, ICC
- Molekulargewicht:
- 160-200 kDa
- Spezies-Reaktivität:
- Human
- Aufreinigung:
- Affinity Purified
- Lagerbedingungen:
- -20[o]C Avoid freeze/thaw cycles.
- Hersteller:
- Abclonal
- Host:
- Rabbit
- Reaktivitäten:
- Human, Mouse, Rat
- Immunogen:
- Synthetic peptide. This information is considered to be commercially sensitive.
- Formulierung:
- Unmodified
- Sequenz:
- MKGSRVERAEEALAHMGSSVFSGITLTKFGGIVVLAFAKSQIFQIFYFRMYLAMVLLGATHGLIFLPVLLSYIGPSVNKAKSCATEERYKGTERERLLNF
- Uniprot:
- O15118
- Synonyme:
- Niemann-Pick C1 protein;NPC;NPC intracellular cholesterol transporter 1;POGZ;SLC65A1;truncated Niemann-Pick C1
- Weitere Details:
- This gene encodes a large protein that resides in the limiting membrane of endosomes and lysosomes and mediates intracellular cholesterol trafficking via binding of cholesterol to its N-terminal domain. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome - the last loop being at the N-terminus. This protein transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by over accumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compartments.
- Versandbedingungen:
- Blue Ice