Niemann-Pick C1 Antibody (NB400-148)

Niemann-Pick C1 Antibody (NB400-148) (0.025 ml)

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	Catalog Number	NB400-148
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anti-Niemann-Pick C1 protein antibody, anti-FLJ98532 antibody, anti-NPC antibody, anti-Niemann-Pick disease, type C1 antibody

Niemann-Pick C1 Antibody Summary:

Species:	Hu, Mu, ChHa	Gene:	NPC1
Applications:	WB, IF, IHC-P, IP	Purity:	Affinity purified
Clonality:	Polyclonal	Host:	Rabbit
Specificity:	This is specific for NPC1 protein.
Publications:	NB400-148 has been mentioned in at least 17 Publications

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Niemann-Pick C1 Antibody Details:

Immunogen:	A synthetic peptide made to the C-terminal region of human NPC1.
Localization:	Late endosome and Lysosome membrane; Single-pass membrane protein.

Species Reactivity:

This antibody reacts with human, mouse, and Chinese hamster protein. Other species have not been tested.

Applications:

Uses:	This antibody is useful for Western blot, detecting heterogeneously glycosylated NPC1 protein with prominent bands at 170 and 220 kDa. It has also been tested for immuno-EM (on human protein only).
Dilutions:	Immunofluorescence 1:250, Immunohistochemistry-Paraffin, Immunoprecipitation, Western Blot 1:1000-1:3000
Unit Size:	0.025 ml (also available: 0.1 ml)
Concentration:	1.0 mg/ml
Positive Controls:	1 Positive Controls

Packaging:

Storage:	Store at 4°C short term. Aliquot and store at -20°C long term. Avoid freeze-thaw cycles.
Buffer:	PBS
Preservative:	0.1% Sodium Azide
Limitations:	This product is for research use only and is not approved for use in humans or in clinical diagnosis. Products are guaranteed for 6 months from date of receipt, except for peptides and proteins which are guaranteed for 3 months.

Gene Symbol: NPC1

Entrez:	4864 (Human)
Entrez:	18145 (Mouse)
Uniprot:	O15118 (Human)

Related Genes:

Background:

Niemann-Pick type C1 (NPC1) is a member of a family of genes encoding membrane-bound proteins containing putative sterol sensing domains. NPC1 protein regulates cholesterol transport from late endosomes-lysosomes to other intracellular compartments. NPC1 overexpression increases the rate of trafficking of low density lipoprotein cholesterol to the endoplasmic reticulum and the rate of delivery of endosomal cholesterol to the plasma membrane. NPC disease is an inherited neurovisceral lipid storage disorder of unesterified cholesterol accumulation in lysosomes. It is characterized by progressive neural and liver degeneration, resulting from inactivating mutations in NPC1, in most cases.

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