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Hunter syndrome among Ashkenazi Jews in Israel; evidence for prenatal selection favoring the Hunter allele

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Summary

Analysis of Ashkenazi families with Hunter patients in Israel demonstrated the complete absence of new mutations among the probands' mothers. Furthermore, in these families a significant deviation of the segregation ratio between the Hunter gene and the normal allele was demonstrated among offspring of heterozygous mothers or siblings of affected children. This may be due to pre- or postzygotic prenatal selection, favoring the X chromosome carrying the Hunter gene among Ashkenazi Jews.

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References

  • Archer IM, Rees DW, Oladimeji A, Wusteman FS, Harper PS (1982) Detection of female carriers of Hunter syndrome; comparison of serum and hair root analysis. J Inherited Metab Dis 5:15–16

    Google Scholar 

  • Chakravarti A, Bale SJ (1983) Differences in the frequency of X-linked deleterious genes in human populations. Am J Hum Genet 35:1252–1257

    PubMed  Google Scholar 

  • Francke U, Felsenstein J, Gartler SM, Migeon BR, Dancis J, Seegmiller JE, Bakay F, Nyan WL (1976) The occurrence of new mutants in the X-linked recessive Lesch-Nyhan syndrome. Am J Hum Genet 28:123–137

    PubMed  Google Scholar 

  • Goodman RM (1979) Genetic disorders among the Jewish people. John Hopkins University Press, Baltimore

    Google Scholar 

  • Haldane JBS (1935) The rate of spontaneos mutation of a human gene. J Genet 31:317–326

    PubMed  Google Scholar 

  • Liebaers I, Di Natale P, Neufeld EF (1977) Iduronate sulfate sulfatase in amniotic fluid; an aid in the prenatal diagnosis of the Hunter syndrome. J Pediatr 90:423–425

    PubMed  Google Scholar 

  • McKusick VA, Neufeld EF (1982) The mucopolysaccharide storage disease. In: Stanburry JB, Wyngaarden JB, Fredrickson DS, Goldstein JL, Brown MS (eds) The metabolic basis of inherited disease, 5th edn. McGraw Hill, New York, pp 751–777

    Google Scholar 

  • Schaap T, Bach G (1980) Incidence of mucopolysaccharidosis in Israel: Is Hunter disease a “Jewish disease”? Hum Genet 56:221–223

    PubMed  Google Scholar 

  • Sherman SL, Morton NE, Jacobs PA, Turner G (1984) The marker (X) syndrome: A cytogenetic and genetic analysis. Ann Hum Genet 48:21–37

    PubMed  Google Scholar 

  • Snedecor GW, Cochran WG (1967) Statistical methods, 6th edn. Iowa State University Press, Ames

    Google Scholar 

  • Statistical Abstracts of Israel (1983) 34, Central Bureau of Statistics

  • Tønnesen T (1984) The use of fructose 1-phosphate to detect Hunter heterozygotes in fibroblast cultures from high risk carriers. Hum Genet 66:212–216

    Article  PubMed  Google Scholar 

  • Vogel F (1984) Mutation and selection in the marker (X) syndrome: A hypothesis. Ann Hum Genet 48:327–332

    PubMed  Google Scholar 

  • Vogel F, Motulsky AG (1979) Human genetics: Problems and approaches. Springer, Berlin Heidelberg New York, pp 301–311

    Google Scholar 

  • Young ID, Harper PS, Archer IM, Newcombe RG (1982) A clinical and genetic study of Hunter syndrome. Heterogeneity. J Med Genet 19:401–407

    PubMed  Google Scholar 

  • Zlotogora J, Bach G (1984) Heterozygote detection in Hunter syndrome. Am J Med Genet 17:661–665

    PubMed  Google Scholar 

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Zlotogora, J., Schaap, T., Zeigler, M. et al. Hunter syndrome among Ashkenazi Jews in Israel; evidence for prenatal selection favoring the Hunter allele. Hum Genet 71, 329–332 (1985). https://doi.org/10.1007/BF00388459

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  • DOI: https://doi.org/10.1007/BF00388459

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