Abstract
Antibodies that are specific for an enzyme may interfere with its catalytic activity: inhibition of the enzyme may occur but activation can also take place. Antibodies raised against certain wild type enzymes of bacteria and fungi have been found to activate some genetic mutants of the same enzymes. We would like to report the first antibody activation of a mutant human enzyme observed in vitro. Extracts of liver biopsy specimens from 5 children with hereditary fructose intolerance (HFI) were examined for fructaldolase activity. Typical reduction of the catalytic activity as well as reduced thermal stability of fructaldolase were demonstrated. When the extracts were reacted with antiserum against human liver fructaldolase B, up to three-fold activation of fructose-1-phosphate aldolase was observed in 3 out of 5 samples. This observation indicates genetic heterogeneity amongst HFI patients and perhaps more importantly, it lends support to the speculation that some day at least in some human mutants engineering of the mutant enzyme may become possible.
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Gitzelmann, R., Steinmann, B. Antibody activation of mutant fructaldolase. Pediatr Res 8, 910 (1974). https://doi.org/10.1203/00006450-197411000-00076
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DOI: https://doi.org/10.1203/00006450-197411000-00076