Abstract
Acute intermittent porphyria (AIP) or precursor syndrome is a well described neuropathic clinical entity with incompletely known etiology. The most prominent biological abnormalities associated with this syndrome are elevations in serum and hepatic δ-aminolevulinic acid (ALA) and porphobilinogen (PBG). We determined the impact of ALA and PBG on human neuroblastoma and glioblastoma tumor cell survival as measured by the MTT assay. ALA proved to be cytotoxic in neuroblastoma cells, while PBG lacked cytotoxic effects. This cytotoxic effect of ALA could be enhanced by deferoxamine and diminished by heme, presumably through modulation of ALA synthesis. In conclusion, ALA excess may prove to be associated with the development of neuropathy in AIP.
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Helson, L., Braverman, S. & Mangiardi, J. δ-Aminolevulinic acid effects on neuronal and glial tumor cell lines. Neurochem Res 18, 1255–1258 (1993). https://doi.org/10.1007/BF00975044
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DOI: https://doi.org/10.1007/BF00975044