Neurochemical aspects of porphyria. Studies on the possible neurotoxicity of delta aminolaevulinic acid

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dc.contributor.author Shanley, B. C.
dc.contributor.author Neethling, A. C.
dc.contributor.author Percy, V. A.
dc.contributor.author Carstens, M.
dc.date.accessioned 2011-03-18T14:57:55Z
dc.date.available 2011-03-18T14:57:55Z
dc.date.issued 1975
dc.identifier.citation Shanley, B.C., Neethling, A.C., Percy, V.A & Carstens, M. 1975, Neurochemical aspects of porphyria. Studies on the possible neurotoxicity of delta aminolaevulinic acid, SA Medical Journal, 49(14), 576-580, http://archive.samj.org.za/
dc.identifier.issn 0256-9574 (print)
dc.identifier.issn 2078-5135 (online)
dc.identifier.uri http://hdl.handle.net/10019.1/7741
dc.description Article
dc.description The original publication is available at http://www.samj.org.za
dc.description.abstract It has been proposed that delta aminolaevulinic acid (ALA), which is overproduced in the inherited hepatic porphyrias, may be responsible for the neurological manifestations of the acute attacks seen in these disorders. Studies were conducted in rats to test the neurotoxicity of ALA. It was found that, after intraperitoneal or subcutaneous injections, ALA is rapidly eliminated via the kidneys. In nephrectomised animals sustained elevation of blood ALA concentration was demonstrated, but despite this, brain uptake was extremely low. Experiments on incorporation of [4 14C] ALA into brain haem yielded similar information. After intraventricular injection of [4 14C] ALA, significant uptake by brain tissue occurred. The subsequent disappearance of ALA was moderately rapid and was virtually complete within 24 hours. Uptake of [4 14C] ALA was apparently significantly greater in the hypothalamus than in other brain areas. The subcellular distribution of radioactivity did not reveal any preferential uptake by nerve endings. Intraventricular injection of unlabelled ALA revealed definite but transitory neurotoxic effects in doses of 3 micromoles and greater. These include involuntary movements and ataxia. No effect of ALA administration on brain protein synthesis could be demonstrated. It is concluded that ALA does have effects on the nervous system in vivo, but the significance of these effects in relation to the pathogenesis of the neurological manifestations of acute porphyria is questionable. en-ZA
dc.description.sponsorship South African Medical Research Council
dc.description.sponsorship Dr A. J. van Wyk
dc.description.sponsorship University of Stellenbosch
dc.description.sponsorship Cape Provincial Administration
dc.format.extent p. 576-580
dc.language.iso en_ZA
dc.publisher Health and Medical Publishing Group (HMPG)
dc.subject Hepatotoxicology en_ZA
dc.subject Rats as laboratory animals en_ZA
dc.subject Hepatic porphyrias -- Diagnosis en_ZA
dc.subject Delta-aminolaevullinic acid en_ZA
dc.subject Porphyrias -- Neurological aspects en_ZA
dc.subject Rats -- Effect of intraventricular injections on en_ZA
dc.subject Neurotoxicity in rats en_ZA
dc.title Neurochemical aspects of porphyria. Studies on the possible neurotoxicity of delta aminolaevulinic acid en_ZA
dc.type Article
dc.description.version Publishers' version
dc.rights.holder Health and Medical Publishing Group (HMPG)


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