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A4795

Niemann Pick C1 Rabbit monoclonal antibody

Size

POA

SKU:
A4795
Additional Names:
Niemann Pick C1|NPC|POGZ|SLC65A1
Application:
ELISA, WB, IHC-P, IF, ICC
Molecular Weight:
160-200 kDa
Species Reactivity:
Human
Purification:
Affinity Purified
Storage Conditions:
-20[o]C Avoid freeze/thaw cycles.
Supplier:
Abclonal
Host:
Rabbit
Reactivities:
Human, Mouse, Rat
Immunogen:
Synthetic peptide. This information is considered to be commercially sensitive.
Formulation:
Unmodified
Sequence:
MKGSRVERAEEALAHMGSSVFSGITLTKFGGIVVLAFAKSQIFQIFYFRMYLAMVLLGATHGLIFLPVLLSYIGPSVNKAKSCATEERYKGTERERLLNF
Uniprot:
O15118
Synonyms:
Niemann-Pick C1 protein;NPC;NPC intracellular cholesterol transporter 1;POGZ;SLC65A1;truncated Niemann-Pick C1
Extra 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.
Shipping Conditions:
Blue Ice