Four cholesterol-sensing proteins

Curr Opin Struct Biol. 1998 Aug;8(4):435-9. doi: 10.1016/s0959-440x(98)80119-x.

Abstract

What is the connection among the following three medical conditions: Niemann-Pick type C disease (a cause of mental retardation and early death), systemic lipidosis (in which an obscure side effect of numerous drugs transforms lysosomes into lamellar bodies), and holoprosencephaly (a catastrophe in embryonic development)? Recent evidence suggests that the pathogenesis in each use involves impaired sensing of cellular cholesterol.

Publication types

  • Review

MeSH terms

  • Carrier Proteins*
  • Cholesterol / metabolism*
  • Holoprosencephaly / metabolism
  • Homeostasis*
  • Humans
  • Hydroxymethylglutaryl CoA Reductases / chemistry
  • Hydroxymethylglutaryl CoA Reductases / metabolism
  • Intracellular Signaling Peptides and Proteins
  • Lipidoses / metabolism
  • Membrane Glycoproteins*
  • Membrane Proteins / chemistry
  • Membrane Proteins / metabolism*
  • Niemann-Pick Diseases / metabolism
  • Patched Receptors
  • Proteins / chemistry
  • Proteins / metabolism
  • Receptors, Cell Surface

Substances

  • Carrier Proteins
  • Intracellular Signaling Peptides and Proteins
  • Membrane Glycoproteins
  • Membrane Proteins
  • NPC1 protein, human
  • Patched Receptors
  • Proteins
  • Receptors, Cell Surface
  • SREBP cleavage-activating protein
  • Cholesterol
  • Hydroxymethylglutaryl CoA Reductases