Pathology of the inferior olivary nucleus in patients with multiple system atrophy

Acta Neuropathol. 2002 Jun;103(6):550-4. doi: 10.1007/s00401-001-0500-x. Epub 2002 Feb 1.

Abstract

The inferior olivary nucleus (ION) from nine patients with multiple system atrophy was examined with antibodies against alpha-synuclein, ubiquitin, synaptophysin, glial fibrillary acidic protein, the Golgi apparatus (GA)-trans-Golgi network (TGN), and microglia/macrophages. As previously reported, there were neuronal loss, gliosis, and alpha-synuclein-positive cytoplasmic inclusions in neurons and glia. In addition, all neurons with alpha-synuclein-positive cytoplasmic inclusions contained abnormal profiles of the GA and TGN, which were reduced in size and numbers. This finding suggests a relationship between the pathogenetic mechanisms causing inclusion body formation and abnormalities of the GA-TGN. This study is also consistent with the conclusion that lesions of the ION may not always reflect changes of transsynaptic degeneration secondary to Purkinje cell loss.

Publication types

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

MeSH terms

  • Aged
  • Female
  • Glial Fibrillary Acidic Protein / metabolism
  • Glycoproteins*
  • Golgi Apparatus / metabolism
  • Golgi Apparatus / pathology*
  • Humans
  • Immunohistochemistry
  • Inclusion Bodies / metabolism
  • Inclusion Bodies / pathology*
  • Male
  • Medulla Oblongata / metabolism
  • Medulla Oblongata / pathology*
  • Medulla Oblongata / physiopathology
  • Membrane Glycoproteins / metabolism
  • Membrane Proteins*
  • Middle Aged
  • Multiple System Atrophy / metabolism
  • Multiple System Atrophy / pathology*
  • Multiple System Atrophy / physiopathology
  • Nerve Tissue Proteins / metabolism
  • Neurons / metabolism
  • Neurons / pathology*
  • Olivary Nucleus / metabolism
  • Olivary Nucleus / pathology*
  • Olivary Nucleus / physiopathology
  • Receptors, Cell Surface / metabolism
  • Receptors, Fibroblast Growth Factor
  • Retrograde Degeneration / metabolism
  • Retrograde Degeneration / pathology*
  • Retrograde Degeneration / physiopathology
  • Sialoglycoproteins / metabolism
  • Synaptophysin / metabolism
  • Synucleins
  • Ubiquitin / metabolism
  • alpha-Synuclein

Substances

  • Glial Fibrillary Acidic Protein
  • Glycoproteins
  • Membrane Glycoproteins
  • Membrane Proteins
  • Nerve Tissue Proteins
  • Receptors, Cell Surface
  • Receptors, Fibroblast Growth Factor
  • SNCA protein, human
  • Sialoglycoproteins
  • Synaptophysin
  • Synucleins
  • TGOLN2 protein, human
  • Ubiquitin
  • alpha-Synuclein
  • cysteine-rich fibroblast growth factor receptor