Phosphorylated high molecular weight neurofilament protein in lower motor neurons in amyotrophic lateral sclerosis and other neurodegenerative diseases involving ventral horn cells

Acta Neuropathol. 1990;79(4):402-8. doi: 10.1007/BF00308716.


Lower motor neurons of the spinal cord of patients with amyotrophic lateral sclerosis (ALS), Werdnig-Hoffmann's disease (WH), X-linked recessive bulbospinal neuronopathy (X-BSNP) and multiple system atrophy (MSA), all of which were known to involve the lower motor neurons, were immunohistochemically examined by using a monoclonal antibody (Ta-51) specific to phosphorylated epitopes of high molecular weight subunits of neurofilaments. The incidence of Ta-51-positive neurons was significantly increased in ALS, WH and MSA, but not in X-BSNP. Ta-51-positive neurons showed a wide variety of morphological appearances, including neurons with normal appearance, central chromatolysis, simple atrophy and neurons containing massive neurofilamentous accumulation. In aged-control cases, similar Ta-51-positive neurons were observed, although to a much lesser extent. In ALS, spheroids and globules, which were strongly positive for Ta-51, were also significantly increased. Ta-51-positive motor neurons, spheroids and globules appeared in proportional to the number of remaining large motor neurons in ALS.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Aged, 80 and over
  • Amyotrophic Lateral Sclerosis / metabolism*
  • Amyotrophic Lateral Sclerosis / pathology
  • Antibodies, Monoclonal
  • Child
  • Child, Preschool
  • Humans
  • Immunohistochemistry
  • Infant
  • Intermediate Filament Proteins / metabolism*
  • Middle Aged
  • Motor Neurons / metabolism*
  • Motor Neurons / pathology
  • Nervous System Diseases / metabolism*
  • Nervous System Diseases / pathology
  • Neurofilament Proteins
  • Spinal Cord / metabolism*
  • Spinal Cord / pathology


  • Antibodies, Monoclonal
  • Intermediate Filament Proteins
  • Neurofilament Proteins