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PCSK9 Molecular Target Information Overview


  • Molecular name: PCSK9, proprotein convertase subtilisin/kexin type 9
  • Gene family: Proprotein convertase subtilisin/kexin family
  • Other names: NARC-1; FH3
  • Former name: HCHOLA3
  • Alias: hypercholesterolemia, autosomal dominant 3


    • PCSK9 molecular target review


    Proprotein convertase subtilisin/kexin type 9 (PCSK9) is a member of the proteinase K subfamily of the secreted subtilisin protease family. It is synthesized as a soluble proenzyme in the endoplasmic reticulum through an intramolecular autocatalytic process. PCSK9 binds to the low-density lipoprotein (LDL) cholesterol receptor and induces LDLR degradation. PCSK9 variants can either lower or increase circulating cholesterol. Other variants are associated with a very rare autosomal dominant form of familial hypercholesterolemia (HCHOLA3). Mutations enhance its protease activity, lower LDLR levels, and prevent cholesterol uptake by cells.


    Human PCSK9 Molecular Target Information


    Molecular name: PCSK9, proprotein convertase subtilisin/kexin type 9


    Alias:

    • convertase subtilisin/kexin type 9 preproprotein
    • FH3
    • FHCL3
    • HCHOLA3
    • LDLCQ1
    • NARC-1
    • NARC1
    • neural apoptosis regulated convertase 1
    • neural apoptosis-regulated convertase 1
    • PC9
    • proprotein convertase 9
    • subtilisin/kexin-like protease PC9

    Gene sequence:NCBI_Gene: 255738

    Protein sequence:UniProtKB: Q8NBP7


    Human PCSK9 target molecular function (prediction)


    Enables several functions, including lipoprotein particle receptor binding activity; serine-type endopeptidase activity; and sodium channel inhibitor activity. Involved in several processes, including cholesterol homeostasis; low-density lipoprotein particle receptor catabolic process; and regulation of receptor-mediated endocytosis. Located in several cellular components, including Golgi apparatus; endosome; and perinuclear region of cytoplasm. Part of PCSK9-AnxA2 complex and PCSK9-LDLR complex. Implicated in familial hypercholesterolemia and hypobetalipoproteinemia.


    Mouse Pcsk9 molecular target information


    Molecular name:Pcsk9, proprotein convertase subtilisin/kexin type 9


    Alias:

    • AI415265
    • AI747682
    • expressed sequence AI415265
    • expressed sequence AI747682
    • Narc1


    Gene sequence:NCBI_Gene: 100102

    Protein sequence:


    Mouse Pcsk9 target molecular function (prediction)


    Acts upstream of or within several processes, including low-density lipoprotein particle receptor particle metabolic process; regulation of low-density lipoprotein particle receptor catabolic process; and triglyceride metabolic process. Located in COPII-coated ER to Golgi transport vesicle; endoplasmic reticulum; and extracellular space. Is expressed in several structures, including alimentary system; cerebellum; genitourinary system; liver; and telencephalon. Human ortholog(s) of this gene implicated in familial hypercholesterolemia and hypobetalipoproteinemia. Orthologous to human PCSK9 (proprotein convertase subtilisin/kexin type 9).


    Rat Pcsk9 molecular target information


    Molecular name:Pcsk9, proprotein convertase subtilisin/kexin type 9

    Alias:

    • NARC-1
    • Narc1
    • neural apoptosis regulated convertase 1
    • neural apoptosis-regulated convertase 1
    • PC9
    • proprotein convertase 9
    • proprotein convertase PC9
    • subtilisin/kexin-like protease PC9

    Gene sequence:NCBI_Gene: 298296

    Protein sequence:UniProtKB: P59996


    Rat Pcsk9 target molecular function (prediction)


    Predicted to enable several functions, including lipoprotein particle binding activity; lipoprotein particle receptor binding activity; and serine-type endopeptidase activity. Involved in several processes, including cellular response to insulin stimulus; cholesterol homeostasis; (proprotein convertase subtilisin/kexin type 9).