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To show liver failure mediated by 5-aminolevulinic acid (ALA), a heme precursor accumulated in inborn and acquired porphyrias, rats were treated with succinylacetone methyl ester (SAME). Treated rats underwent the expected ALA accumulation in plasma, liver and urine as a result from inhibition of ALA dehydratase (ALAD) activity. The enzyme activity decreased concomitantly with diminished urinary coproporphyrin levels. Additionally, liver protein carbonyls, iron and ferritin were higher in groups treated with a lower concentration of SAME whereas malondialdehyde was higher in the group treated with a higher ester dose. Consistent with these biochemical data, chronic treatment SAME was associated with induced oxidative subcellular and tissue damage revealed by cytosolic and mitochondrial changes within the liver cells. Altogether, these data expand the understanding of the direct biochemical mechanism for liver cell death in hepatic inborn disorders by generating excess ALA and may foster future therapeutic-driven strategies to preserve liver function.

Liver Damage Induced by Succinylacetone: A Shared Redox Imbalance Mechanism between Tyrosinemia and Hepatic Porphyrias