Aquaporin-4 overexpression in rat ALS model

Anat Rec (Hoboken). 2009 Feb;292(2):207-13. doi: 10.1002/ar.20838.

Abstract

Onset of motoneuron death characterizing amyotrophic lateral sclerosis (ALS) is closely linked to modified astrocytic and glial environments. Here, we show that in the spinal cord from transgenic rat overexpressing mutated human SOD1, aquaporin-4 mRNA and protein are specifically overexpressed in the gray matter at end stage of disease. Immunohistochemistry and double immunofluorescence allowed to detect, in the spinal cord gray matter of the ALS rat, increased aquaporin-4 surrounding both vessel and motoneuron perikarya. The use of pre-embedding immunohistochemistry at electron microscopic level confirmed such localization associated with swollen astrocytic processes surrounding the vessels. The AQP4 immunohistochemical labeling surrounding several motoneuron perikarya was only seen in ALS rats. Identification of this AQP4-positive cellular type remains unclear.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Amino Acid Substitution
  • Amyotrophic Lateral Sclerosis / genetics*
  • Amyotrophic Lateral Sclerosis / metabolism*
  • Amyotrophic Lateral Sclerosis / pathology
  • Animals
  • Animals, Genetically Modified
  • Aquaporin 4 / genetics*
  • Aquaporin 4 / metabolism*
  • Astrocytes / metabolism
  • Astrocytes / pathology
  • Base Sequence
  • DNA Primers / genetics
  • Disease Models, Animal
  • Humans
  • Immunohistochemistry
  • Microscopy, Fluorescence
  • Microscopy, Immunoelectron
  • Motor Neurons / metabolism
  • Motor Neurons / pathology
  • Mutagenesis, Site-Directed
  • RNA, Messenger / genetics
  • RNA, Messenger / metabolism
  • Rats
  • Rats, Sprague-Dawley
  • Recombinant Proteins / genetics
  • Recombinant Proteins / metabolism
  • Spinal Cord / blood supply
  • Spinal Cord / metabolism*
  • Spinal Cord / pathology
  • Superoxide Dismutase / genetics
  • Superoxide Dismutase / metabolism
  • Superoxide Dismutase-1

Substances

  • Aqp4 protein, rat
  • Aquaporin 4
  • DNA Primers
  • RNA, Messenger
  • Recombinant Proteins
  • SOD1 protein, human
  • Sod1 protein, rat
  • Superoxide Dismutase
  • Superoxide Dismutase-1