|Immunohistochemistry (IHC)||5-10 µg/ml|
|Immunohistochemistry (Paraffin) (IHC (P))||5-10 µg/ml|
|Western Blot (WB)||1:1000-1:3000|
|Immunoprecipitation (IP)||See 1 publications below|
|Tested Species reactivity||Hamster, Human, Mouse, Non-human primate, Pig, Rat|
|Published species reactivity||Human|
|Host / Isotype||Rabbit / IgG|
|Immunogen||A synthetic peptide made to the C-terminal region of human NPC1.|
|Contains||0.1% sodium azide|
|Storage conditions||Store at 4°C short term. For long term storage, store at -20°C, avoiding freeze/thaw cycles.|
By Western blot, PA1-16817 detects heterogeneously glycosylated NPC1 protein with prominent bands at ~170 and ~220 kDa.
Suggested positive control: human fibroblast cell lysate.
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.
For Research Use Only. Not for use in diagnostic procedures. Not for resale without express authorization.
Protein Aliases: FLJ98532; Niemann Pick type C1; Niemann-Pick C disease protein; Niemann-Pick C1 protein; Niemann-Pick disease, type C1; Niemann-Pick type C1; NPC; sphingomyelinosis; truncated Niemann-Pick C1
Gene Aliases: A430089E03Rik; C85354; D18Ertd139e; D18Ertd723e; I79_014078; lcsd; nmf164; NPC; NPC1; spm
Molecular Function: receptor
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