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July 14, 1993, Vol 270, No. 2 >
ARTICLE | July 14, 1993

Oncology FREE

Judith E. Karp, MD; Samuel Broder, MD
JAMA. 1993;270(2):237-239. doi:10.1001/jama.1993.03510020105035
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References

Few individuals have had as much influence on our thinking about human heredity and development as the Austrian monk, Gregor Mendel. When we teach medical genetics, we are in effect updating Mendel's principles. According to Mendel, for autosomal genes, a gene is a gene is a gene; that is, all copies of a given gene are expressed equally. Although most of our observations regarding patterns of inheritance follow the principles of mendelian genetics, there is a growing list of discrete areas where observation diverges from expectation, ie, nonmendelian genetics come into play. With the advent of sophisticated molecular technology, we can dissect the origins and patterns of transmission of specific genes, adding an unprecedented dimension to our understanding of disease inheritance and the factors that determine net genomic expression. This may have special relevance for understanding the development of cancer, many forms which are not inherited in a straightforward manner

REFERENCES

1 +
Ferguson-Smith AC, Reik W, Surani MA.  Genomic imprinting and cancer. Cancer Surv . 1990;;9:487-503.
2 +
Reik W.  Genomic imprinting and genetic disorders in man. Trends Genet . 1989;;5:331-336.
3 +
Mascari MJ, Gottlieb W, Rogan PK, et al.  The frequency of uniparental disomy in Prader-Willi syndrome. N Engl J Med . 1992;; 326:1599-1607.
4 +
Engel E.  A new genetic concept: uniparental disomy and its potential effect, isodisomy. Am J Med Genet . 1980;;6:137-143.
5 +
Cattanach BM, Barr JA, Evans EP, et al.  A candidate mouse model for Prader-Willi syndrome which shows an absence of Snrpn expression. Nature Genet . 1992;;2:270-274.
6 +
Leff SE, Brannan CI, Reed ML, et al.  Maternal imprinting of the mouse Snrpn gene and conserved linkage homology with the human Prader-Willi syndrome. Nature Genet . 1992;;2:259-264.
7 +
Ozcelik T, Leff S, Robinson W, et al.  Small nuclear ribonucleoprotein polypeptide N (SNRPN), an expressed gene in the Prader-Willi syndrome critical region. Nature Genet . 1992;;2:265-269.
8 +
Junien C.  Beckwith-Wiedemann syndrome, tumorigenesis and imprinting. Curr Opin Genet Dev . 1992;;2:431-438.
9 +
Brown KW, Gardner A, Williams JC, Mott MG, McDermott A, Maitland NJ.  Paternal origin of 11p15 duplications in the Beckwith-Wiedemann syndrome. Cancer Genet Cytogenet . 1992;;58:66-70.
10 +
Henry I, Bonaiti-Pellie C, Chehensse V, et al.  Uniparental paternal disomy in a genetic cancer-predisposing syndrome. Nature . 1991;;351:665-667.
11 +
Koufos A, Grundy P, Morgan K, et al.  Familial Wiedemann-Beckwith syndrome and a second Wilms tumor locus both map to 11p15.5. Am J Hum Genet . 1989;;44:711-719.
12 +
Ping AJ, Reeve AE, Law DJ, Young MR, Boehnke M, Feinberg AP.  Genetic linkage of Beckwith-Wiedemann syndrome to 11p15. Am J Hum Genet . 1989;;44:720-723.
13 +
Viljoen D, Ramesar R.  Evidence for paternal imprinting in familial Beckwith-Wiedemann syndrome. J Med Genet . 1992;;29:221-225.
14 +
Maw MA, Grundy PE, Millow LJ, et al.  A third Wilms' tumor locus on chromosome 16q. Cancer Res . 1992;;52:3094-3098.
15 +
Haas OA, Argyriou-Tirita A, Lion T.  Parental origin of chromosomes involved in the translocation t(9;22). Nature . 1992;;359: 414-416.
16 +
Reik W.  Imprinting in leukemia. Nature . 1992;;359:362-363.

Figures

Tables

Interactive Graphics

Video

Country-Specific Mortality and Growth Failure in Infancy and Yound Children and Association With Material Stature

Use interactive graphics and maps to view and sort country-specific infant and early dhildhood mortality and growth failure data and their association with maternal

1 +
Ferguson-Smith AC, Reik W, Surani MA.  Genomic imprinting and cancer. Cancer Surv . 1990;;9:487-503.
2 +
Reik W.  Genomic imprinting and genetic disorders in man. Trends Genet . 1989;;5:331-336.
3 +
Mascari MJ, Gottlieb W, Rogan PK, et al.  The frequency of uniparental disomy in Prader-Willi syndrome. N Engl J Med . 1992;; 326:1599-1607.
4 +
Engel E.  A new genetic concept: uniparental disomy and its potential effect, isodisomy. Am J Med Genet . 1980;;6:137-143.
5 +
Cattanach BM, Barr JA, Evans EP, et al.  A candidate mouse model for Prader-Willi syndrome which shows an absence of Snrpn expression. Nature Genet . 1992;;2:270-274.
6 +
Leff SE, Brannan CI, Reed ML, et al.  Maternal imprinting of the mouse Snrpn gene and conserved linkage homology with the human Prader-Willi syndrome. Nature Genet . 1992;;2:259-264.
7 +
Ozcelik T, Leff S, Robinson W, et al.  Small nuclear ribonucleoprotein polypeptide N (SNRPN), an expressed gene in the Prader-Willi syndrome critical region. Nature Genet . 1992;;2:265-269.
8 +
Junien C.  Beckwith-Wiedemann syndrome, tumorigenesis and imprinting. Curr Opin Genet Dev . 1992;;2:431-438.
9 +
Brown KW, Gardner A, Williams JC, Mott MG, McDermott A, Maitland NJ.  Paternal origin of 11p15 duplications in the Beckwith-Wiedemann syndrome. Cancer Genet Cytogenet . 1992;;58:66-70.
10 +
Henry I, Bonaiti-Pellie C, Chehensse V, et al.  Uniparental paternal disomy in a genetic cancer-predisposing syndrome. Nature . 1991;;351:665-667.
11 +
Koufos A, Grundy P, Morgan K, et al.  Familial Wiedemann-Beckwith syndrome and a second Wilms tumor locus both map to 11p15.5. Am J Hum Genet . 1989;;44:711-719.
12 +
Ping AJ, Reeve AE, Law DJ, Young MR, Boehnke M, Feinberg AP.  Genetic linkage of Beckwith-Wiedemann syndrome to 11p15. Am J Hum Genet . 1989;;44:720-723.
13 +
Viljoen D, Ramesar R.  Evidence for paternal imprinting in familial Beckwith-Wiedemann syndrome. J Med Genet . 1992;;29:221-225.
14 +
Maw MA, Grundy PE, Millow LJ, et al.  A third Wilms' tumor locus on chromosome 16q. Cancer Res . 1992;;52:3094-3098.
15 +
Haas OA, Argyriou-Tirita A, Lion T.  Parental origin of chromosomes involved in the translocation t(9;22). Nature . 1992;;359: 414-416.
16 +
Reik W.  Imprinting in leukemia. Nature . 1992;;359:362-363.

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