Immunoreactivities of p62, an ubiqutin-binding protein, in the spinal anterior horn cells of patients with amyotrophic lateral sclerosis

J Neurol Sci. 2006 Nov 1;249(1):13-8. doi: 10.1016/j.jns.2006.05.060. Epub 2006 Jul 3.

Abstract

An ubiquitin-binding protein, p62, is one of the components of the ubiquitin-containing inclusions in several human neurodegenerative diseases. Amyotrophic lateral sclerosis (ALS) is characterized by the presence of skein-like inclusions, Lewy body-like inclusions, and basophilic inclusions in the remaining anterior horn cells, in which these inclusions contain ubiquitin, while the other characteristic inclusions of Bunina type are ubiquitin-negative. We examined the spinal cord from 28 ALS cases including two ALS with dementia and two ALS with basophilic inclusions, using antibody to p62. The results demonstrated that p62 localized in skein-like inclusions, Lewy body-like inclusions and basophilic inclusions. The number of p62-positive inclusions observed in the remaining anterior horn cells of each section was variable among the ALS cases. In contrast, Bunina bodies, that do not contain ubiquitin, were negative for p62. As far as we examined, the 11 non-ALS cases did not show any p62 immunoreactivities in the anterior horn cells. Our results suggested that p62 plays important roles in forming the inclusions and may be associated with the protection of the neurons from degenerative processes involving ubiquitin.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adaptor Proteins, Signal Transducing
  • Adult
  • Amyotrophic Lateral Sclerosis / diagnosis
  • Amyotrophic Lateral Sclerosis / metabolism*
  • Amyotrophic Lateral Sclerosis / physiopathology
  • Anterior Horn Cells / metabolism*
  • Anterior Horn Cells / pathology
  • Anterior Horn Cells / physiopathology
  • Female
  • Humans
  • Immunohistochemistry
  • Inclusion Bodies / metabolism*
  • Inclusion Bodies / pathology
  • Lewy Bodies / metabolism
  • Lewy Bodies / pathology
  • Male
  • Middle Aged
  • Nerve Degeneration / metabolism*
  • Nerve Degeneration / physiopathology
  • Predictive Value of Tests
  • Proteins / immunology
  • Proteins / metabolism*
  • Sequestosome-1 Protein
  • Spinal Cord / metabolism
  • Spinal Cord / pathology
  • Spinal Cord / physiopathology
  • Staining and Labeling / methods
  • Ubiquitin / metabolism*

Substances

  • Adaptor Proteins, Signal Transducing
  • Proteins
  • SQSTM1 protein, human
  • Sequestosome-1 Protein
  • Ubiquitin