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Vector Laboratories, Inc.
6737 Mowry Ave
Newark, CA 94560
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NPC / NPC1
Niemann-Pick disease, type C1
Intracellular cholesterol transporter which acts in concert with NPC2 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. Binds oxysterol with higher affinity than cholesterol. May play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals.
| Gene Name: | Niemann-Pick disease, type C1 |
| Synonyms: | NPC1, NPC, Niemann-Pick C1 protein, Niemann-Pick disease, type C1 |
| Target Sequences: | NM_000271 |
Publications (18)
1
Niemann-pick type C1 (NPC1) overexpression alters cellular cholesterol homeostasis. Millard EE, Srivastava K, Traub LM, Schaffer JE, Ory DS. The Journal of biological chemistry. 2000 275:38445-51. (ICC, WB)
[PubMed:10964915]
2
Cholesterol overload promotes morphogenesis of a Niemann-Pick C (NPC)-like compartment independent of inhibition of NPC1 or HE1/NPC2 function. Frolov A, Srivastava K, Daphna-Iken D, Traub LM, Schaffer JE, Ory DS. The Journal of biological chemistry. 2001 276:46414-21. (ICC)
[PubMed:11571306]
3
Purified NPC1 protein. I. Binding of cholesterol and oxysterols to a 1278-amino acid membrane protein. Infante RE, Abi-Mosleh L, Radhakrishnan A, Dale JD, Brown MS, Goldstein JL. The Journal of biological chemistry. 2008 283:1052-63. (WB; Human)
[PubMed:17989073]
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NPC / NPC1 Mouse anti-Human Monoclonal (17B5) Antibody
Human
IF, IHC, IHC-P, IP, WB
Unconjugated
50 µg/$485
NPC / NPC1 Mouse anti-Human Monoclonal (HRP) Antibody
Human
IF, WB
HRP Conjugated
100 µl/$1,104
NPC / NPC1 Mouse anti-Human Monoclonal (FITC) Antibody
Human
IF, WB
FITC Conjugated
100 µl/$1,104
NPC / NPC1 Mouse anti-Human Monoclonal (PE) Antibody
Human
IF, WB
PE Conjugated
100 µl/$1,104
NPC / NPC1 Mouse anti-Human Monoclonal (APC) (17B5) Antibody
Human
IF, WB
APC Conjugated
100 µl/$1,104
NPC / NPC1 Mouse anti-Human Monoclonal (aa34-174) (8D10B6) Antibody
Human
ELISA, IHC, IHC-P, WB
Unconjugated
100 µl/$459
NPC / NPC1 Rabbit anti-Human Polyclonal (aa591-620) Antibody
Human
ELISA, IF, IHC, WB
Unconjugated
200 µl/$734
NPC / NPC1 Rabbit anti-Human Polyclonal (aa591-620) (HRP) Antibody
Human
ELISA, IHC, WB
HRP Conjugated
200 µl/$952
NPC / NPC1 Rabbit anti-Human Polyclonal (aa591-620) (Biotin) Antibody
Human
ELISA, IF, IHC, WB
Biotin Conjugated
200 µl/$952
NPC / NPC1 Rabbit anti-Human Polyclonal (aa591-620) (FITC) Antibody
Human
ELISA, ICC, IF, IHC, WB
FITC Conjugated
200 µl/$952
NPC / NPC1 Rabbit anti-Human Polyclonal (aa591-620) (APC) Antibody
Human
ELISA, ICC, IF, IHC, WB
APC Conjugated
200 µl/$952
NPC / NPC1 Rabbit anti-Human Polyclonal (aa591-620) (PE) Antibody
Human
ELISA, ICC, IF, IHC, WB
PE Conjugated
200 µl/$952
NPC / NPC1 Mouse anti-Human Monoclonal (4H2) Antibody
Human
ELISA, IHC, IHC-P, WB
Unconjugated
50 µg/$460
NPC / NPC1 Rabbit anti-Human Polyclonal Antibody
Mouse, Human
ELISA, IF, IHC, IHC-P, WB
Unconjugated
50 µg/$460
NPC / NPC1 Rabbit anti-Human Polyclonal (C-Terminus) Antibody
Mouse, Hamster, Primate, Human
IF, IHC, IHC-P, IP, WB
Unconjugated
50 µl/$460
NPC / NPC1 Rabbit anti-Human Polyclonal (C-Terminus) (Biotin) Antibody
Mouse, Hamster, Human
IF, IHC, IHC-P, IP, WB
Biotin Conjugated
0.1 ml/$416
NPC / NPC1 Rabbit anti-Human Polyclonal (C-Terminus) (DY650) Antibody
Mouse, Hamster, Human
IF, IHC, IHC-P, IP, WB
DY650 Conjugated
0.1 ml/$416
NPC / NPC1 Rabbit anti-Human Polyclonal (C-Terminus) (DY488) Antibody
Mouse, Human
IF, IHC, IHC-P, IP, WB
DY488 Conjugated
0.1 ml/$416
NPC / NPC1 Rabbit anti-Human Polyclonal (C-Terminus) (DY550) Antibody
Mouse, Hamster, Human
IF, IHC, IHC-P, IP, WB
DY550 Conjugated
0.1 ml/$416
NPC / NPC1 Goat anti-Mouse Polyclonal (aa1039-1052) Antibody
Mouse
Peptide-ELISA
Unconjugated
100 µg/$401
NPC / NPC1 Rabbit anti-Human Polyclonal (C-Terminus) Antibody
Mouse, Human
IF, IHC, IP, WB
Unconjugated
100 µl/$649
Sandwich
96-Well Microplate
Human
0.312 - 20 ng/ml
Colorimetric - 450nm (TMB)
Cell Lysates, Tissue Homogenates
1 Plate/$654
HEK 293 Cells
His-DDK (Flag)
Less than 1.0 EU/µg protein (determined by LAL method).
32 kDa
100 µg/$843
NPC / NPC1 Rabbit anti-Human Polyclonal Antibody
Human
ELISA, IHC, WB
Unconjugated
100 µg/$351
NPC / NPC1 Rabbit anti-Human Polyclonal Antibody
Human
IHC-P, WB
Unconjugated
50 µg/$294; 100 µg/$360
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