Fragmentation of the Golgi apparatus of the ballooned neurons in patients with corticobasal degeneration and Creutzfeldt-Jakob disease

Acta Neuropathol. 2000 Sep;100(3):270-4. doi: 10.1007/s004010000182.


We investigated immunohistologically the Golgi apparatus (GA) and the trans-Golgi network (TGN) of the cortical ballooned neurons (BNs) in two patients with corticobasal degeneration and in five with Creutzfeldt-Jakob disease. We observed that the BNs showed fragmentation of the GA and the TGN, accompanied by a reduction in the number of fragmented Golgi elements and by a unique perinuclear distribution. This is the first report of the abnormalities of GA in cortical BNs. These findings suggests that the GA and the TGN may play some roles in the BN formation.

Publication types

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

MeSH terms

  • Aged
  • Cerebral Cortex / metabolism
  • Cerebral Cortex / pathology*
  • Cerebral Cortex / physiopathology
  • Creutzfeldt-Jakob Syndrome / metabolism
  • Creutzfeldt-Jakob Syndrome / pathology*
  • Creutzfeldt-Jakob Syndrome / physiopathology
  • Female
  • Glycoproteins*
  • Golgi Apparatus / metabolism
  • Golgi Apparatus / pathology*
  • Humans
  • Male
  • Membrane Glycoproteins / metabolism
  • Membrane Proteins*
  • Middle Aged
  • Nerve Degeneration / metabolism
  • Nerve Degeneration / pathology*
  • Nerve Degeneration / physiopathology
  • Neurons / metabolism
  • Neurons / pathology*
  • Receptors, Cell Surface*
  • Receptors, Fibroblast Growth Factor
  • Sialoglycoproteins / metabolism


  • Glycoproteins
  • Membrane Glycoproteins
  • Membrane Proteins
  • Receptors, Cell Surface
  • Receptors, Fibroblast Growth Factor
  • Sialoglycoproteins
  • TGOLN2 protein, human
  • cysteine-rich fibroblast growth factor receptor