Letter | Published:

Maternal histidine metabolism and its effect on foetal development in the mouse


THERE have been reports1,2 of possible deleterious effects on the mental development of children due to maternal histidinaemia, in which a deficiency of the enzyme histidase leads to the exposure of the foetus to abnormally high concentrations of histidine and its imidazole derivatives. This is analagous to the well established case of maternal phenylketonuria3 which can lead to mental retardation in metabolically normal children. There is also a suggested link4 between low maternal concentrations of two enzymes in galactose metabolism and infantile cataracts. We report here work on the nature of the maternal effects of histidinaemia using mutant mice which are the homologues of the human condition5–7.

Access optionsAccess options

Rent or Buy article

Get time limited or full article access on ReadCube.


All prices are NET prices.


  1. 1

    Lyon, I. C. T., Gardner, R. J. M., and Veale, A. M. O., Arch. Dis. Child., 49, 581 (1974).

  2. 2

    Harper, P. S., Heredity, 34, 289 (1975).

  3. 3

    Denniston, J. C., J. Pediat., 63, 461 (1963).

  4. 4

    Harley, J. D., Mutton, P., Irvine, S., and Gupta, J. A., Lancet, ii, 259 (1974).

  5. 5

    Kacser, H., Bulfield, G., and Wallace, M. E., Nature, 244, 77 (1973).

  6. 6

    Bulfield, G., and Kacser, H., Arch. Dis. Child., 49, 545 (1974).

  7. 7

    Bulfield, G., and Kacser, H., J. Neurochem., 24, 403 (1975).

  8. 8

    Rugh, R., Radiology, 82, 917 (1964).

  9. 9

    Blake, R. L., and Russell, E. S., Science, 176, 809 (1972).

  10. 10

    Blake, R. L., Biochem. J., 129, 987 (1972).

  11. 11

    Blake, R. L., and Russell, E. S., Genetics, 74, 526 (1973).

  12. 12

    McLaren, A., and Michie, D., J. exp. Biol., 33, 394 (1956).

  13. 13

    Wallace, M. E., Mouse Newslett., 42, 20 (1970).

  14. 14

    Wallace, M. E., Mouse Newslett., 48, 25 (1973).

Download references

Author information

Rights and permissions

Reprints and Permissions

About this article

Further reading


By submitting a comment you agree to abide by our Terms and Community Guidelines. If you find something abusive or that does not comply with our terms or guidelines please flag it as inappropriate.