Laronidase-functionalized multiple-wall lipid-core nanocapsules: promising formulation for a more effective treatment of mucopolysaccharidosis type I

Pharm Res. 2015 Mar;32(3):941-54. doi: 10.1007/s11095-014-1508-y. Epub 2014 Sep 11.

Abstract

Purpose: Mucopolysaccharidosis I is a genetic disorder caused by alpha-L-iduronidase deficiency. Its primary treatment is enzyme replacement therapy (ERT), which has limitations such as a high cost and a need for repeated infusions over the patient's lifetime. Considering that nanotechnological approaches may enhance enzyme delivery to organs and can reduce the dosage thereby enhancing ERT efficiency and/or reducing its cost, we synthesized laronidase surface-functionalized lipid-core nanocapsules (L-MLNC).

Methods: L-MLNCs were synthesized by using a metal complex. Size distributions were evaluated by laser diffraction and dynamic light scattering. The kinetic properties, cytotoxicity, cell uptake mechanisms, clearance profile and biodistribution were evaluated.

Results: Size distributions showed a D[4,3] of 134 nm and a z-average diameter of 71 nm. L-MLNC enhanced the Vmax and Kcat in comparison with laronidase. L-MLNC is not cytotoxic, and nanocapsule uptake by active transport is not only mediated by mannose-6-phosphate receptors. The clearance profile is better for L-MLNC than for laronidase. A biodistribution analysis showed enhanced enzyme activity in different organs within 4 h and 24 h for L-MLNC.

Conclusions: The use of lipid-core nanocapsules as building blocks to synthesize surface-functionalized nanocapsules represents a new platform for producing decorated soft nanoparticles that are able to modify drug biodistribution.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Area Under Curve
  • Biological Transport
  • Cell Survival / drug effects
  • Cells, Cultured
  • Chemistry, Pharmaceutical
  • Enzyme Replacement Therapy* / adverse effects
  • Fibroblasts / drug effects*
  • Fibroblasts / metabolism
  • Fibroblasts / pathology
  • Iduronidase / administration & dosage
  • Iduronidase / chemistry*
  • Iduronidase / genetics
  • Iduronidase / pharmacokinetics
  • Iduronidase / toxicity
  • Injections, Intravenous
  • Lipids / chemistry*
  • Metabolic Clearance Rate
  • Mice, Knockout
  • Mucopolysaccharidosis I / drug therapy*
  • Mucopolysaccharidosis I / enzymology
  • Nanocapsules*
  • Nanomedicine
  • Particle Size
  • Technology, Pharmaceutical / methods
  • Tissue Distribution

Substances

  • Lipids
  • Nanocapsules
  • Iduronidase