Ubiquitin in motor neuron disease: study at the light and electron microscope.

摘要: Several neurodegenerative diseases, including motor neuron disease (MND), are characterized by formation of abnormal cytoskeleton-derived inclusions which contain ubiquitin (Ubq). We have studied the distribution Ubq in 26 cases MND with light and electron microscopic immunocytochemistry. Ubiquitin-positive were found neurons anterior horns most amyotrophic lateral sclerosis (ALS) but not present other forms MND. Ubiquitin immunoreactivity was observed 10-15 nm intraneuronal filaments, stained antibodies to neurofilaments, on dense bodies dystrophic neurites throughout neuropil pyramidal tracts. Data analysis showed a trend toward lower percentage Ubq-positive longer duration illness or number neurons. A high occurred an aggressive clinical course, suggesting that ubiquitination takes place at early stages disease.