An investigation of pyruvate metabolism in patients with cerebellar and spinocerebellar degeneration

I.R. Livingstone; F.L. Mastaglia; R.J.T. Pennington

doi:10.1016/0022-510X(80)90155-0

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An investigation of pyruvate metabolism in patients with cerebellar and spinocerebellar degeneration

Journal article

Peer reviewed

An investigation of pyruvate metabolism in patients with cerebellar and spinocerebellar degeneration

I.R. Livingstone, F.L. Mastaglia and R.J.T. Pennington

Journal of the Neurological Sciences, Vol.48(1), pp.123-132

1980

DOI: https://doi.org/10.1016/0022-510X(80)90155-0

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Abstract

This study extends previous observations of pyruvate metabolism in the spinocerebellar degenerations by screening for abnormalities of pyruvate oxidation using the rise in blood pyruvate after an oral glucose load and examining the activity of the lipoamide dehydrogenase (LAD) moeity of the pyruvate dehydrogenase complex in the serum of 31 patients with Friedreich's ataxia, hereditary spastic ataxia and primary cerebellar degeneration. Serum LAD activity was significantly reduced in 10 Friedreich's ataxia patients when compared to controls and to 10 patients with spastic ataxia, thus confirming previous studies. Two patients with Friedreich's ataxia and 2 with primary cerebellar degeneration had abnormal blood pyruvate curves after oral glucose loading. The findings suggest that abnormal pyruvate oxidation occurs in some cases of Friedreich's ataxia and primary cerebellar degeneration and that the abnormality of pyruvate metabolism is not necessarily reflected in the serum LAD activity of these patients. The relevance of these findings to the heterogeneity of the hereditary ataxias is discussed.

Details

Title: An investigation of pyruvate metabolism in patients with cerebellar and spinocerebellar degeneration
Authors/Creators: I.R. Livingstone (Author/Creator)
F.L. Mastaglia (Author/Creator)
R.J.T. Pennington (Author/Creator)
Publication Details: Journal of the Neurological Sciences, Vol.48(1), pp.123-132
Publisher: Elsevier BV
Identifiers: 991005544238907891
Murdoch Affiliation: Murdoch University
Language: English
Resource Type: Journal article

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Collaboration types: Domestic collaboration; International collaboration
Citation topics: 1 Clinical & Life Sciences; 1.52 Neurodegenerative Diseases; 1.52.951 Huntington's and Ataxias
Web Of Science research areas: Clinical Neurology; Neurosciences
ESI research areas: Neuroscience & Behavior