Research noteNeurotoxicity of δ-aminolevulinic acid and porphobilinogen
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Cited by (28)
The acute porphyrias
2020, Rosenberg’s Molecular and Genetic Basis of Neurological and Psychiatric Disease: Volume 1Porphyria-induced posterior reversible encephalopathy syndrome and central nervous system dysfunction
2019, Molecular Genetics and MetabolismCitation Excerpt :The most widely accepted hypothesis suggests that ALA produced excessively in the liver is the main pathogenic factor [5]. ALA can inhibit or activate γ-aminobutyric acid receptors and is also pro-oxidative so that it may lead to neuro and cytotoxicity [6,7]. The fact that liver transplantation stops recurrences of attacks and normalizes the urinary excretion of porphyrin precursors supports that the liver is the primary pathogenic site.
Porphyric neuropathy
2013, Handbook of Clinical NeurologyCitation Excerpt :Attacks may be triggered by medications, hormonal changes, stress, and starvation (Kauppinen and Mustajoki, 1992; De Siervi et al., 1999; Hift and Meissner, 2005). These stressors may induce an attack either through direct induction of aminolevulinate synthase (ALAS), which leads to an increase in the hepatic production of neurotoxic metabolites such as ALA and PBG, or by increasing the demand for heme synthesis in the liver (Pierach and Edwards, 1978; Meyer et al., 1998; Podvinec et al., 2004). The list of medications that may induce a porphyric attack is extensive and includes a number of anticonvulsant drugs, antihypertensive medications, and antibiotics (Gorchein, 1997; American Porphyria Foundation, 2010).
5-Aminolevulinate and 4, 5-dioxovalerate ions decrease GABA<inf>A</inf> receptor density in neuronal cells, synaptosomes and rat brain
2006, Brain ResearchCitation Excerpt :It has been shown that ALA accumulation induces convulsions (Emanuelli et al., 2000), for ALA toxicity also involves glutamatergic pathways, since ALA irreversibly inhibits glutamate uptake in astrocytes through inhibition of the GLT-1 glutamate transporter (Emanuelli et al., 2003). Moreover, ALA damages GABAA receptors (Demasi et al., 1996), and acts as a GABA antagonist (Pierach and Edwards, 1978), besides stimulating glutamate release (Brennan and Cantrill, 1979). This suggests that ALA may be involved in neuronal cell death due to calcium influx (Choi, 1994).
Effects of 5-aminolevulinic acid on the glutamatergic neurotransmission
2003, Neurochemistry International
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The authors thank Irene Bossenmaier, Brett Gemlo, and Harry Draeger for their excellent collaboration.