Rabbit polyclonal antibody to NPC1 [Niemann-Pick type C1]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.
Immunogen
A synthetic peptide made to the C-terminal region of human NPC1.
Key Feature
Clonality
Polyclonal
Host Species
Rabbit
Tested Applications
ICC/IFIHC-PIPWB
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 immunofluorescence, immunoprecipitation, and immuno-EM (on human protein only). No other applications have been tested. Suggested Starting Dilutions*:Western Blot: 1:1,000-1:3,000 (ECL)Immunofluorescence: 1:250 Immunoprecipitation: Investigator must determine*Optimal working dilutions should be determined by the investigator.:
Species Reactivity
HumanMouseHamster
Concentration
1mg/ml
Target Information
Gene Synonyms
FLJ98532 NPC
Target
NPC1
Tissue Specificity
This antibody is specific for NPC1 protein.
Application
Application
Immunohistochemistry-Paraffin: Niemann-Pick C1 Antibody [ - Staining of human brain, cortex, neurons and astrocytes.
Application
Western Blot: Niemann-Pick C1 Antibody [ - Detection of human NPC1 in 20 ug of human fibroblast cell lysate.
Positive Control
Human fibroblast cell lysate
Application Notes
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 immunofluorescence, immunoprecipitation, and immuno-EM (on human protein only). No other applications have been tested. Suggested Starting Dilutions*:Western Blot: 1:1,000-1:3,000 (ECL)Immunofluorescence: 1:250 Immunoprecipitation: Investigator must determine*Optimal working dilutions should be determined by the investigator.:
Additional Information
Form
Liquid
Storage Instructions
Keep as concentrated solution. Store at 4ºC short term. For extended storage aliquot and store at -20ºC or below. Avoid freeze-thaw cycles.
Storage Buffer
0.1 ml of affinity purified antibody in PBS containing 0.025% sodium azide.