Niemann Pick C1/NPC1 Proteins, Antibodies, cDNA Clones Research Reagents

NPC1 (NPC Intracellular Cholesterol Transporter 1, also known as NPC; POGZ; SLC65A1), located on 18q11.2, is conserved in chimpanzee, Rhesus monkey, dog, cow, mouse, rat, chicken, zebrafish, fruit fly, mosquito, S.cerevisiae, K.lactis, E.gossypii, M.oryzae, N.crassa, A.thaliana, and frog. The gene produces a 142167 Da protein composed of 1278 amino acids. This gene encodes a large protein that resides in the limiting membrane of endosomes and lysosomes and mediates intracellular cholesterol trafficking via binding of cholesterol to its N-terminal domain. Diseases such as Niemann-Pick Disease, Type C1, and Headache are associated with NPC1. The related pathways of NPC1 include Lipoprotein metabolism and Lysosome.

Niemann Pick C1/NPC1 Protein (1)

    Niemann Pick C1/NPC1 Antibody (2)

      Niemann Pick C1/NPC1 cDNA Clone (1)


      In expression vector

      Niemann Pick C1/NPC1 Lysate (1)

        Niemann Pick C1/NPC1 の背景知識

        Niemann-Pick C1 (NPC1), a host receptor involved in the envelope glycoprotein (GP)-mediated entry of filoviruses into cells, is believed to be a major determinant of cell susceptibility to filovirus infection. Niemann-Pick C1 (NPC1), a membrane protein of lysosomes, is required for the export of cholesterol derived from receptor-mediated endocytosis of LDL. The NPC1 protein is a multipass transmembrane protein whose deficiency causes the autosomal recessive lipid storage disorder Niemann-Pick type C1. NPC1 localizes predominantly to late endosomes and has a dileucine motif located within a small cytoplasmic tail thought to target the protein to this location. Niemann-Pick disease type C1 (NPC1) is a rare progressive neurodegenerative disorder caused by mutations in the NPC1 gene. On the cellular level, NPC1 mutations lead to an accumulation of cholesterol and gangliosides.

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