Letter | Published:

A Biochemical Lesion in Congenital, Non-obstructive, Non-hæmolytic Jaundice

Naturevolume 180pages14261427 (1957) | Download Citation



CONGENITAL, non-obstructive, non-hæmolytic jaundice is a syndrome characterized by marked elevation of ‘indirect-reacting’ (free) bilirubin in the blood1. Recent studies have shown that bilirubin forms a polar glucuronide which is readily excreted in the bile1. The synthesis of bilirubin glucuronide has been found to be catalysed by an enzyme system in the microsomes of the liver that transfers glucuronic acid from uridine diphosphate glucuronic acid to a Suitable aglycone2. We wish to describe experiments showing that the accumulation of free bilirubin in this syndrome is the result of a defect in the glucuronide-forming mechanism.

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  1. 1

    (a) Malloy, H. T., and Loewenstein, L., Canad. Med. Assoc. J., 42, 122 (1940). (b) Crigler, T. F., and Najjar, V. A., Pediatrics, 10, 169 (1952). (c) Schmid, R., Science, 124, 76 (1956). (d) Billing, B. H., and Lathe, G. H., Biochem. J., 63, 6P (1956).

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    Schmid, R., Hammacker, L., and Axelrod, J., Arch. Biochem. Biophys., 70, 285 (1957).

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    Strominger, J. L., Maxwell, E. S., Axelrod, J., and Kalckar, H. M., J. Biol. Chem., 224, 79 (1957).

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    Brodie, B. B., and Axelrod, J., J. Pharmacol. and Exp. Therap., 94, 22 (1948).

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    Peterson, R. E., and Schmid, R., J. Clin. Endocrinol. (in the press).

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  1. National Institute of Mental Health

  2. National Institute of Arthritis and Metabolic Diseases, National Institutes of Health, Bethesda, 14, Md.



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