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Recombinant Human NPC intracellular cholesterol transporter 1 (NPC1), Truncated

ACP01869

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Specifications


Cat.No ACP01869 Target NameNPC1
Target SynonymsNiemann-Pick C1 proteinFormLiquid or Lyophilized powder
Expression SystemYeastExpression Range23-261aa
Mol Weight30.2 kDaProtein LengthPartial
PurityGreater than 90% as determined by SDS-PAGE.Storage Buffer5%-50% glycerol. Lyophilized powder form: the buffer before lyophilization is Tris/PBS-based buffer, 6% Trehalose, Liquid form: default storage buffer is Tris/PBS-based buffer, pH 8.0.

Immunogen Information


Target SpeciesHumanUniprot IDO15118
Background Information
  • Uniprot Id

    O15118

  • Target Species

    Human

  • Target Name

    NPC1

  • Target Full Name

    NPC intracellular cholesterol transporter 1

  • Target Function

    Intracellular cholesterol transporter which acts in concert with NPC2 and plays an important role in the egress of cholesterol from the endosomal/lysosomal compartment. Unesterified cholesterol that has been released from LDLs in the lumen of the late endosomes/lysosomes is transferred by NPC2 to the cholesterol-binding pocket in the N-terminal domain of NPC1. Cholesterol binds to NPC1 with the hydroxyl group buried in the binding pocket. Binds oxysterol with higher affinity than cholesterol. May play a role in vesicular trafficking in glia, a process that may be crucial for maintaining the structural and functional integrity of nerve terminals (Probable).; (Microbial infection) Acts as an endosomal entry receptor for ebolavirus.

  • Target Involvement

    Niemann-Pick disease C1 (NPC1)

  • Target Subcellular Location

    Late endosome membrane; Multi-pass membrane protein. Lysosome membrane; Multi-pass membrane protein.

  • Target Protein Families

    Patched family

  • Target Research Area

    Cardiovascular, Transcription

  • Target Synonyms

    Niemann Pick C1 protein precursor; Niemann Pick disease, type C1; Niemann-Pick C1 protein; NPC; NPC1; NPC1_HUMAN

  • Target Background

    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. It is predicted to have a cytoplasmic C-terminus, 13 transmembrane domains, and 3 large loops in the lumen of the endosome - the last loop being at the N-terminus. This protein transports low-density lipoproteins to late endosomal/lysosomal compartments where they are hydrolized and released as free cholesterol. Defects in this gene cause Niemann-Pick type C disease, a rare autosomal recessive neurodegenerative disorder characterized by over accumulation of cholesterol and glycosphingolipids in late endosomal/lysosomal compartments.

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