Abstract
The mitochondrial 4977-bp common deletion has been reported in some studies to occur
exclusively or with increased frequency in the midbrain of patients with Parkinson's
disease (PD). Other studies could not confirm these results; rather, it was suggested
that the mitochondrial common deletion is associated with aging in the midbrain and
not PD. One possible explanation for these conflicting results is the difficulty in
quantifying mitochondrial DNA deletions or mutations in the whole midbrain or substantia
nigra (SN) while only a subset of midbrain neurons degenerate in PD. In addition,
none of the studies has addressed the cell types with the common deletion within the
midbrain. In this study we used in situ hybridization to detect the common deletion
in sections of midbrain from patients with PD, multiple system atrophy-parkinsonian
type (MSA-P), progressive supranuclear palsy (PSP), dementia with Lewy bodies (DLB),
age-matched controls, and individuals of different ages. The results demonstrated
that the mitochondrial common deletion accumulated primarily in neurons but not glia
in both the SN and other midbrain regions. There was no significant difference in
the number or distribution of neurons with the common deletion or the average of the
mean densities (AMD) of staining with the common deletion in nigral neurons among
patients with PD, MSA-P, PSP, DLB, or age-matched controls. In addition, there was
no difference in the number or distribution of neurons with the common deletion in
nigral neurons between any age group, although there was a tendency for the common
deletion to increase in the non-nigral neurons in older patients. These data indicate
that accumulation of the 4977-bp common deletion in mitochondrial DNA in midbrain
occurred primarily in neurons, and by this cytological approach, it was not associated
with nigral neurodegeneration in the common movement disorders or aging.
Keywords
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Article info
Publication history
Accepted:
June 25,
2001
Identification
Copyright
© 2002 Published by Elsevier Inc.
