Abstract
Extract: AH enzymes of the urea cycle are demonstrable in circulating leukocytes. They show the same relative activities as those in liver except for argininosuccinate synthetase + lyase (combined) which seems to be disproportionately active.
To see whether leukocytes reflect liver activity, blood from patients with three hepatic urea cycle disorders was tested. In each case, the leukocytes showed the same enzyme deficiency as was apparent from a liver biopsy (Table 4). Leukocyte assays appear to be reliable indicators of the enzyme lesions in inherited urea cycle enzyme defects and therefore may obviate the need for liver biopsy.
Speculation: By using radioactive substrates to improve sensitivity it should be possible to reduce the blood sample size required (from 30 ml for the less active enzymes) to amounts suitable for use in neonates. Heterozygote detection in relatives of affected patients may also be feasible.
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Wolfe, D., Gatfield, P. Leukocyte Urea Cycle Enzymes in Hyperammonemia. Pediatr Res 9, 531–535 (1975). https://doi.org/10.1203/00006450-197506000-00004
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DOI: https://doi.org/10.1203/00006450-197506000-00004
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