3-Nitropropionic acid increases frataxin expression in human lymphoblasts and in transgenic rat PC12 cells

Neurosci Lett. 2003 Oct 30;350(3):184-6. doi: 10.1016/s0304-3940(03)00906-6.

Abstract

Friedreich ataxia (FRDA) is the most common recessive ataxia caused by reduced expression of frataxin, a nuclear encoded mitochondrial protein. In this study we examined the effects of 3-nitropropionic acid (3-NP) on frataxin expression in FRDA patient and control lymphoblasts and in rat pheochromocytoma cell line (PC12) overexpressing human frataxin. Our studies showed an up-regulation of frataxin expression in both FRDA and control lymphoblasts following exposure to 3-NP. In addition, in transgenic frataxin overexpressing cells 3-NP caused an increase of frataxin protein.

Publication types

  • Comparative Study
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Animals
  • Blotting, Western
  • Dose-Response Relationship, Drug
  • Enzyme Inhibitors / pharmacology*
  • Frataxin
  • Friedreich Ataxia / metabolism
  • Humans
  • Iron-Binding Proteins / biosynthesis*
  • Iron-Binding Proteins / drug effects*
  • Iron-Binding Proteins / genetics
  • Lymphocytes / drug effects*
  • Nitro Compounds
  • Oxidative Stress
  • PC12 Cells
  • Propionates / pharmacology*
  • Rats
  • Stem Cells / drug effects
  • Time Factors
  • Transgenes

Substances

  • Enzyme Inhibitors
  • Iron-Binding Proteins
  • Nitro Compounds
  • Propionates
  • 3-nitropropionic acid