NPC1 Antibody (G-1): sc-271335

NPC1 Antibody (G-1) is a mouse monoclonal IgA kappa light chain antibody that detects NPC1 protein of mouse, rat, and human origin by western blotting (WB), immunoprecipitation (IP), immunofluorescence (IF), and enzyme-linked immunosorbent assay (ELISA). anti-NPC1 antibody (G-1) is available as the non-conjugated form. NPC1 plays a crucial role in intracellular cholesterol trafficking, residing primarily in late endosomes and lysosomes, which is vital for maintaining cellular cholesterol homeostasis. Cholesterol is essential for various cellular functions, including membrane structure, signaling, and steroid hormone synthesis. The human NPC1 gene, located on chromosome 18q11.2, encodes a protein that undergoes N-glycosylation, which is important for stability and function. NPC1 contains a cysteine-rich domain that is critical for proper functioning, yet this region is highly susceptible to mutations. Such mutations in NPC1 are responsible for Niemann-Pick disease type C (NPC), an autosomal recessive disorder characterized by unesterified cholesterol accumulation within the endosomal/lysosomal system, leading to severe neurodegeneration and neurological symptoms, including hepatosplenomegaly, ataxia, dystonia, and dementia. With over 90% of NPC cases attributed to mutations in NPC1, studying NPC1 is essential for understanding disease pathophysiology and developing potential therapeutic strategies.

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NPC1 Antibody (G-1) References:

1. Mutations in the leucine zipper motif and sterol-sensing domain inactivate the Niemann-Pick C1 glycoprotein.  |  Watari, H., et al. 1999. J Biol Chem. 274: 21861-6. PMID: 10419504
2. Mutations in NPC1 highlight a conserved NPC1-specific cysteine-rich domain.  |  Greer, WL., et al. 1999. Am J Hum Genet. 65: 1252-60. PMID: 10521290
3. A model for niemann-pick type C disease in the nematode Caenorhabditis elegans.  |  Sym, M., et al. 2000. Curr Biol. 10: 527-30. PMID: 10801441
4. The structure and function of the Niemann-Pick C1 protein.  |  Ioannou, YA. 2000. Mol Genet Metab. 71: 175-81. PMID: 11001808
5. Fate of endogenously synthesized cholesterol in Niemann-Pick type C1 cells.  |  Cruz, JC. and Chang, TY. 2000. J Biol Chem. 275: 41309-16. PMID: 11013265
6. Neurons in Niemann-Pick disease type C accumulate gangliosides as well as unesterified cholesterol and undergo dendritic and axonal alterations.  |  Zervas, M., et al. 2001. J Neuropathol Exp Neurol. 60: 49-64. PMID: 11202175
7. Niemann-Pick C variant detection by altered sphingolipid trafficking and correlation with mutations within a specific domain of NPC1.  |  Sun, X., et al. 2001. Am J Hum Genet. 68: 1361-72. PMID: 11349231
8. Establishment and characterization of immortalized Schwann cells from murine model of Niemann-Pick disease type C (spm/spm).  |  Watabe, K., et al. 2001. J Peripher Nerv Syst. 6: 85-94. PMID: 11446388
9. Reovirus infection is regulated by NPC1 and endosomal cholesterol homeostasis.  |  Ortega-Gonzalez, P., et al. 2022. PLoS Pathog. 18: e1010322. PMID: 35263388
10. Species-specific differences in NPC1 protein trafficking govern therapeutic response in Niemann-Pick type C disease.  |  Schultz, ML., et al. 2022. JCI Insight. 7: PMID: 36301667
11. NPC1-dependent alterations in KV2.1-CaV1.2 nanodomains drive neuronal death in models of Niemann-Pick Type C disease.  |  Casas, M., et al. 2023. Nat Commun. 14: 4553. PMID: 37507375
12. EXTL3 and NPC1 are mammalian host factors for Autographa californica multiple nucleopolyhedrovirus infection.  |  Huang, Y., et al. 2024. Nat Commun. 15: 7711. PMID: 39231976

Monoclonal antibodies for mammalian and non-mammalian protein targets represent essentially all targets covered by polyclonal antibodies. Over 8,300 of our antibodies are offered as ImmunoCruz^® conjugates which eliminates the need for a secondary antibody.

Santa Cruz Biotechnology antibodies have over 360,000 research citations. Anti-NPC1 Antibody (G-1) has 2 citations in a variety of scientific publications.

Primary antibodies like Anti-NPC1 Antibody (G-1) for mammalian target proteins are recommended for the detection of a range of mammalian species, primarily of mouse, rat and human species. Many mammalian antibodies are also ideal for veterinary research and reactive with ovine, porcine, caprine, feline, canine, bovine and equine protein targets. Santa Cruz Biotechnology primary monoclonal antibodies are suitable for laboratory research applications and are not intended for diagnostic or therapeutic use.

NPC1 Antibody (G-1) Specifications

Alternate Product Name: NPC1

Product Clone Name: G-1

Classification: Primary Antibody

Clonality: Monoclonal Antibody

Antibody Isotype: IgA kappa light chain

Amino Acids: 516-630

Epitope Target: NPC1

Epitope Species: human

Product Reactivity/Detection: NPC1

Species Reactivity/Detection: mouse, rat, human

Additional Mammalian Reactivity: N/A

Additional Non-Mammalian Reactivity: N/A

Applications: WB, IP, IF, ELISA

Vialing Information: Each vial contains 200 µg IgA kappa light chain in 1.0 ml of PBS with < 0.1% sodium azide and 0.1% gelatin

Research Citations: 2 citations in various scientific publications

Available Conjugates/Forms for NPC1 Antibody (G-1): N/A