This study was performed to explore the potential role of mitochondrial DNA
mutations in the neurodegenerative process in amyotrophic lateral sclerosi
s (ALS). Using a semiquantitative assay, a common mitochondrial DNA deletio
n mutation (mt DNA(4977)) was assayed in brain tissue obtained from six spo
radic ALS patients and compared to four controls. In each brain, levels of
this mutation were measured in a brain region affected by neurodegeneration
, the motor cortex (Brodmann area 4), and compared to the temporal cortex (
Brodmann area 17). In the ALS but not control brains, levels of mt DNA(4977
) were an average of more than 30-fold (range 15-250) higher in Brodmann ar
ea 4. These results support and extend those of previous studies implying t
hat mitochondria may participate in the neurodegenerative process in ALS. N
euroReport 11:2507-2509 (C) 2000 Lippincott Williams & Wilkins.