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NPC2

Niemann-Pick disease, type C2

Intracellular cholesterol transporter which acts in concert with NPC1 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment. Both NPC1 and NPC2 function as the cellular 'tag team duo' (TTD) to catalyze the mobilization of cholesterol within the multivesicular environment of the late endosome (LE) to effect egress through the limiting bilayer of the LE. NPC2 binds unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes and transfers it to the cholesterol-binding pocket of the N-terminal domain of NPC1. Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket and is exported from the limiting membrane of late endosomes/ lysosomes to the ER and plasma membrane by an unknown mechanism. The secreted form of NCP2 regulates biliary cholesterol secretion via stimulation of ABCG5/ABCG8-mediated cholesterol transport.

Gene Name: Niemann-Pick disease, type C2
Synonyms: NPC2, EDDM1, HE1, Epididymal protein 1, NP-C2, Niemann-Pick disease, type C2
Target Sequences: NM_006432 NP_006423.1 P61916

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Products
Proteins (9)
Over-Expression Lysate (2)
Recombinant (7)
NPC2 (9)
No (9)
6His-SUMO, N-terminus (1)
GST (1)
GST, N-terminus (1)
His (3)
His-GST (1)
Myc-DDK (Flag) (2)
Dog (3)
Human (4)
Rat (1)
293T Cells (1)
E. coli (3)
HEK 293 Cells (1)
Human Cells (1)
Wheat Germ Extract (2)
Yeast (1)
Purified (2)
Low endotoxin level (1)
NPC2 Protein - 12.5% SDS-PAGE of human NPC2 stained with Coomassie Blue
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Wheat Germ Extract
GST
42.35 kDa
10 µg/$479; 25 µg/$670
NPC2 Protein - 12.5% SDS-PAGE Stained with Coomassie Blue.
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Wheat Germ Extract
GST, N-terminus
10 µg/$479; 25 µg/$670
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E. coli
His
18.1 kD
1 mg/$1,679; 100 µg/$598; 20 µg/$405
NPC2 Protein
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Yeast
His
16 kD
100 µg/$679; 20 µg/$429; 1 mg/$1,973
NPC2 Protein - Recombinant  Niemann Pick Disease Type C2 By SDS-PAGE
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E. coli
His-GST
44.6 kDa
10 µg/$278; 50 µg/$422; 100 µg/$672; 200 µg/$842; 1 mg/$1,934; 500 µg/$1,480; 5 mg/$2,941; 2 mg/$2,119
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Human Cells
His
Less than 1.0 EU/µg protein (determined by LAL method).
15.9 kDa
100 µg/$843
NPC2 Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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293T Cells
Myc-DDK (Flag)
16.57 kDa
20 µg/$150
NPC2 Protein - Western validation with an anti-DDK antibody * L: Control HEK293 lysate R: Over-expression lysate
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HEK 293 Cells
Myc-DDK (Flag)
16.57 kDa
100 µg/$494
NPC2 Protein - (Tris-Glycine gel) Discontinuous SDS-PAGE (reduced) with 5% enrichment gel and 15% separation gel.
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E. coli
6His-SUMO, N-terminus
1 mg/$1,679; 100 µg/$598; 20 µg/$405
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PLEASE NOTE

For RESEARCH USE ONLY. Intended for use by laboratory professionals. Not intended for human diagnostic or therapeutic purposes.

The data on this page has been compiled from LifeSpan internal sources, the National Center for Biotechnology Information (NCBI), and The Universal Protein Resource (UniProt).