• Publications
  • Influence
Niemann-Pick C1 disease gene: homology to mediators of cholesterol homeostasis.
Transfection of NP-C fibroblasts with wild-type NPC1 cDNA resulted in correction of their excessive lysosomal storage of LDL cholesterol, thereby defining the critical role of NPC1 in regulation of intracellular cholesterol trafficking.
The intracellular transport of low density lipoprotein-derived cholesterol is defective in Niemann-Pick type C fibroblasts
The results indicate that a cholesterol transport defect exists in NPC that is specific for LDL-derived cholesterol.
Modulation of Hormone-sensitive Lipase and Protein Kinase A-mediated Lipolysis by Perilipin A in an Adenoviral Reconstituted System*
Estimation of the relative contributions of Peri A and HSL in basal and PKA-mediated lipolysis in NIH 3T3 fibroblasts found that perilipin expression and phosphorylation state are critical regulators of lipid storage and hydrolysis in ACS1/FATP1 cells.
Evidence for a Cholesterol Transport Pathway from Lysosomes to Endoplasmic Reticulum That Is Independent of the Plasma Membrane*
Evidence is presented that supports a vesicular model of cholesterol transport from lysosomes to the endoplasmic reticulum that is independent of the plasma membrane and is normal in a Chinese hamster ovary mutant with defective plasma membrane-to-ACAT movement.
Niemann–Pick Type C Mutations Cause Lipid Traffic Jam
Recent studies indicating that NPC1 is not a cholesterol‐specific transport molecule are discussed, finding that it appears to be required for the vesicular shuttling of both lipids and fluid‐phase constituents from multivesicular late endosomes to destinations such as the trans‐Golgi network.
Intracellular cholesterol transport.