An immunohistochemical study on alpha-ketoglutarate dehydrogenase complex in Parkinson's disease.

Abstract:

:We report an immunohistochemical study of the mitochondrial alpha-ketoglutarate dehydrogenase complex (KGDHC) in the substantia nigra in Parkinson's disease. The KGDHC, the three enzyme complex catalyzing the oxidation of alpha-ketoglutarate to succinate through succinic semialdehyde, is the rate-regulating enzyme of the TCA cycle. The mitochondrial toxin, MPP+, inhibits not only complex I but also the KGDHC. Therefore, we investigated this enzyme complex in Parkinson's disease. In the control patients (n = 6), the immunostaining of the melanized nigral neurons was generally uniform; most of the melanized neurons showed good immunostaining with some neurons showing somewhat reduced staining. In Parkinson's disease (n = 9), many melanized neurons showed reduced immunostaining for the KGDHC, and those neurons were more frequently seen in the lateral one-third of substantia nigra. The decrease in the immunostaining for the KGDHC correlated roughly with the severity of degeneration. The KGDHC is more vulnerable to degeneration than complex II, III, and IV as noted in our previous immunohistochemical study. Even if secondary, the loss may play a role in the progression of the disease.

journal_name

Ann Neurol

journal_title

Annals of neurology

authors

Mizuno Y,Matuda S,Yoshino H,Mori H,Hattori N,Ikebe S

doi

10.1002/ana.410350212

subject

Has Abstract

pub_date

1994-02-01 00:00:00

pages

204-10

issue

2

eissn

0364-5134

issn

1531-8249

journal_volume

35

pub_type

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