Movement disorders in the Hfe knockout mouse

Nutr Neurosci. 2005 Aug;8(4):239-44. doi: 10.1080/10284150500277685.

Abstract

The Hfe(- /-) mouse is a model for human hereditary hemochromatosis (HHH). The accumulation of tissue iron in this condition has led to the suggestion that HHH patients may be at higher risk for neurodegenerative diseases. In this study, adult male Hfe(-/-) mice and wildtype controls (n = 12/group) were evaluated for impairment with motor tests (stride length, landing footsplay, rotarod) as well as a general observational battery (Functional Observational Battery, FOB). Hfe(-/-) mice were characterized by more falls from the rotarod, wider forelimb landing footsplay and hypersensitivity to proximal stimulation. Iron accumulation in brain was not detected by histopathology. These data suggest that a motor syndrome may be associated with HHH that could be further understood through the Hfe(-/-) mouse model.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Animals
  • Brain / pathology
  • Hemochromatosis / genetics
  • Hemochromatosis Protein
  • Histocompatibility Antigens Class I / physiology
  • Humans
  • Male
  • Membrane Proteins / deficiency*
  • Membrane Proteins / physiology
  • Mice
  • Mice, Inbred C57BL
  • Mice, Knockout
  • Motor Activity
  • Movement Disorders / genetics*
  • Movement Disorders / pathology
  • Mutation

Substances

  • Hemochromatosis Protein
  • Hfe protein, mouse
  • Histocompatibility Antigens Class I
  • Membrane Proteins