Summary
1. Recessive spastic paraplegia is due to a partially sex-linked gene.
2. Dominant spastic paraplegia is due to an autosomal gene. Partially sex-linked recessives are probably responsible for a small fraction of all cases of Friedreich’s ataxia and spastic ataxia.
3. There are probably three or more allelomorphs of the partially sex-linked gene, determining different ages of onset.
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References
Bell, J. (1939). “Hereditary ataxia and spastic paraplegia.”Treas. Hum. Inher. 4, Partiii.
Fisher, R. A. (1936). “Tests of significance applied to Haldane’s data on partial sex-linkage.”Ann. Eugen., Lond.,7, 87–104.
Haldane, J. B. S. (1936). “A search for incomplete sex-linkage in man.”Ann. Eugen., Lond.,7, 28–57.
-- (1940). “The relative importance of principal and modifying genes in determining some human diseases.”J. Genet. (in the Press).
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Haldane, J.B.S. The partial sex-linkage of recessive spastic paraplegia. Journ. of Genetics 41, 141–147 (1941). https://doi.org/10.1007/BF02983017
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DOI: https://doi.org/10.1007/BF02983017