Choline acetylase and glutamic acid decarboxylase in Huntington's chorea

McGeer, P. L.; McGeer, E.G.; Fibiger, H.C.

doi:10.1212/wnl.23.9.912

Cited by 167 publications

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“…In Huntington's disease, G A D activity is generally decreased in the striatum and choline acetyltransferase activity may also be depressed, though to a lesser degree [4, 14,16]. Neither of these neurochemical changes is specific for a single disease, however, because of the wide variability in activity of these enzymes in both control populations and tissue from patients with Huntington's disease [ 181.…”

Section: Discussionmentioning

confidence: 99%

Familial degeneration of the basal ganglia with acanthocytosis: A clinical, neuropathological, and neurochemical study

Bird

Cederbaum

Valpey

et al. 1978

Annals of Neurology

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Three siblings, offspring of normal consanguineous parents, had a progressive neurological disorder characterized primarily by chorea and leading to death in the fourth or fifth decade. The most carefully studied patient had neither malabsorption nor absent serum beta-lipoprotein but did have erythrocyte acanthocytosis. Postmortem examination showed marked neuronal loss and gliosis of the caudate nucleus and putamen. Activities of glutamic acid decarboxylase and choline acetyltransferase were normal in cortex, caudate, and putamen. Autosomal recessive inheritance, acanthocytosis, and probable peripheral neuropathy help differentiate this disorder from Huntington's disease.

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Section: Discussionmentioning

confidence: 99%

Familial degeneration of the basal ganglia with acanthocytosis: A clinical, neuropathological, and neurochemical study

Bird

Cederbaum

Valpey

et al. 1978

Annals of Neurology

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“…Activities of acetylcholine and choline acetyltransferase, which are responsible for cognitive functions, have been reported to be reduced in the brain of HD patients (McGeer and Fibiger, 1973). Cognitive effects of angiotensin II are well documented.…”

Section: Groups Remarksmentioning

confidence: 99%

Potential of protease inhibitor in 3-nitropropionic acid induced Huntington's disease like symptoms: Mitochondrial dysfunction and neurodegeneration

et al. 2014

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“…This latter group of patients with Huntington's disease with a decreased accumulation of HVA, also has been F L Cunha, C R Oliveira, M Diniz, R Amaral, A F Gongalves, and J Pio-Abreu reported to exhibit an increased incidence of paranoid psycosis.38 However, there was no correlation of CSF HVA with the severity of abnormal movements suggesting that factors other than DA metabolism, including alteration of other neurotransmitters, such as acetylcholine and 'y-aminobutyric acid and substance P, may be responsible for the severity of chorea. [39][40][41][42] In Sydenham's chorea, a fast disappearing disease, the reduction of accumulation of HVA after probenecid again suggests a decrease in DA turnover.…”

Section: Discussionmentioning

confidence: 99%

Homovanilic acid in Huntington's disease and Sydenham's chorea.

Cunha¹,

Oliveira²,

Diniz³

et al. 1981

Journal of Neurology, Neurosurgery & Psychiatry

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SUMMARY Homovanilic acid (HVA) was determined in the lumbar CSF of 12 patients with Huntington's disease and 12 with Sydenham's chorea before and after probenecid administration. The means of HVA concentration (basal and after probenecid) were lower in those with Huntington's disease than in controls, and were even lower in a sub-group characterised by increased tone and slowness of voluntary movement. There was no correlation between CSF HVA values and the severity of abnormal movements, nor with length of the illness and age of the patients with Huntington's disease. The mean basal HVA concentration did not differ from controls in those with Sydenham's chorea but the accumulation with probenecid was significantly lower. These results suggest a decrease in cerebral dopamine release in both forms of chorea.About one third of the homovanilic acid (HVA) derived from the catabolism of dopamine (DA) and released in the striatum enters the CSF in the lateral ventricle.' 2 There is some evidence to suggest that the concentration of this acid metabolite in ventricular CSF, is related to the concentration in the striatum.24 But the concentration of HVA cisternal and lumbar CSF is lower, and the correlation with striatal levels disappears.' 5 6 At least three mechanisms are responsible for the lower HVA levels-lumbar CSF (1) the diffusion of the HVA "jet," (2) the CSF circulation, (3) an active transport mechanism localised in the fourth ventricle and sub-arachnoid space.7-8 This active transport mechanism can be blocked with probenecid, which prevents the efflux of HVA and 5-HIAA from CSF.8-1" There has been considerable variation in the dose and timing of probenecid administration to man.'2"15

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Choline acetylase and glutamic acid decarboxylase in Huntington's chorea

Cited by 167 publications

References 0 publications

Familial degeneration of the basal ganglia with acanthocytosis: A clinical, neuropathological, and neurochemical study

Familial degeneration of the basal ganglia with acanthocytosis: A clinical, neuropathological, and neurochemical study

Potential of protease inhibitor in 3-nitropropionic acid induced Huntington's disease like symptoms: Mitochondrial dysfunction and neurodegeneration

Homovanilic acid in Huntington's disease and Sydenham's chorea.

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