Skip to main content

Thank you for visiting You are using a browser version with limited support for CSS. To obtain the best experience, we recommend you use a more up to date browser (or turn off compatibility mode in Internet Explorer). In the meantime, to ensure continued support, we are displaying the site without styles and JavaScript.

Miller-Dieker lissencephaly gene encodes a subunit of brain platelet-activating factor

An Erratum to this article was published on 04 August 1994


PLATELET-ACTIVATING factor (PAF) is involved in a variety of biological and pathological processes1 and PAF acetylhydrolase, which inactivates PAF by removing the acetyl group at the sn-2 position, is widely distributed in plasma and tissue cytosols2,3. One isoform of PAF acetylhydrolase present in bovine brain cortex is a heterotrimer comprising subunits with relative molecular masses of 45K, 30K and 29K (ref. 4). We have now isolated the comple-mentary DNA for the 45K subunit. Sequence analysis revealed a striking identity (99%) of the subunit with a protein encoded by the causative gene (LIS-1) for Miller-Dieker lissencephaly5, a human brain malformation manifested by a smooth cerebral surface and abnormal neuronal migration. This indicates that the LIS-1 gene product is a human homologue of the 45K subunit of intracellular PAF acetylhydrolase. Our results raise the possibility that PAF and PAF acetylhydrolase are important in the formation of the brain cortex during differentiation and development.

This is a preview of subscription content

Access options

Rent or Buy article

Get time limited or full article access on ReadCube.


All prices are NET prices.


  1. 1

    Hanahan, D. J. A. Rev. Biochem. 55, 483–509 (1986).

    CAS  Article  Google Scholar 

  2. 2

    Stafforini, D. M., Prescott, S. M. & Mclntyre, T. M. J. biol. Chem. 262, 4223–4230 (1987).

    CAS  PubMed  Google Scholar 

  3. 3

    Farr, R. S., Cox, C. P., Wardlow, M. L. & Jorgensen, R. Clin. Immun. Immunopath. 15, 318–330 (1980).

    CAS  Article  Google Scholar 

  4. 4

    Hattori, M., Arai, H. & Inoue, K. J. biol. Chem. 288, 18748–18753 (1993).

    Google Scholar 

  5. 5

    Reiner, O. et al. Nature 364, 717–721 (1993).

    ADS  CAS  Article  Google Scholar 

  6. 6

    Shaw, G. & Kamen, R. Cell 46, 659–667 (1986).

    CAS  Article  Google Scholar 

  7. 7

    van dern Voorn, L. & Ploegh, H. L. FEBS Lett. 507, 131–134 (1992).

    Article  Google Scholar 

  8. 8

    Ledbetter, S. A., Kuwano, A., Dobyns, W. B. & Ledbetter, D. H. Am. J. hum. Genet. 50, 182–189 (1992).

    CAS  PubMed  PubMed Central  Google Scholar 

  9. 9

    Dynlacht, B. D., Weinzierl, R. O. J., Admon, A. & Tjian, R. Nature 363, 176–179 (1993).

    ADS  CAS  Article  Google Scholar 

  10. 10

    Sogos, V., Bussolino, F., Pilia, E., Torelli, S. & Gremo, F. J. Neurosci. Res. 27, 706–711 (1990).

    CAS  Article  Google Scholar 

  11. 11

    Honda, Z. et al. Nature 349, 342–345 (1991).

    ADS  CAS  Article  Google Scholar 

  12. 12

    Bito, H. et al. Neuron 9, 285–294 (1992).

    CAS  Article  Google Scholar 

  13. 13

    Catalan, R. E. et al. Biochem. biophys. Res. Commun. 183, 300–305 (1992).

    CAS  Article  Google Scholar 

  14. 14

    Kato, K., Clark, G. D., Bazen, N. G. & Zorumski, C. F. Nature 367, 175–179 (1994).

    ADS  CAS  Article  Google Scholar 

  15. 15

    Dobyns, W. B., Reiner, O., Carrozo, R. & Ledbetter, D. H. J. Am. med. Ass. 270, 2838–2842 (1993).

    CAS  Article  Google Scholar 

  16. 16

    Kornecki, E. & Ehrlich, Y. H. Science 240, 1792–1794 (1988).

    ADS  CAS  Article  Google Scholar 

  17. 17

    Isola, N. R., Harn, H. J. & Cooper, D. L. BioTechniques 11, 580–582 (1991).

    CAS  PubMed  Google Scholar 

  18. 18

    Kwiatkowski, T. J., Zoghbi, H. Y., Ledbetter, S. A., Ellison, K. A. & Chinault, A. C. Nucleic Acids Res. 18, 7191–7192 (1990).

    CAS  Article  Google Scholar 

Download references

Author information



Rights and permissions

Reprints and Permissions

About this article

Cite this article

Hattori, M., Adachi, H., Tsujimoto, M. et al. Miller-Dieker lissencephaly gene encodes a subunit of brain platelet-activating factor. Nature 370, 216–218 (1994).

Download citation

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.


Quick links

Nature Briefing

Sign up for the Nature Briefing newsletter — what matters in science, free to your inbox daily.

Get the most important science stories of the day, free in your inbox. Sign up for Nature Briefing