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      Blaustein’s Pathology of the Female Genital Tract 

      Embryology of the Female Genital Tract and Disorders of Abnormal Sexual Development

      , ,

      Springer New York

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          Most cited references 49

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          DNA binding activity of recombinant SRY from normal males and XY females.

          The protein encoded by the human testis determining gene, SRY, contains a high mobility group (HMG) box related to that present in the T cell-specific, DNA-binding protein TCF-1. Recombinant SRY protein was able to bind to the same core sequence AACAAAG recognized by TCF-1 in a sequence dependent manner. In five XY females point mutations were found in the region encoding the HMG box. In four cases DNA binding activity of mutant SRY protein was negligible; in the fifth case DNA binding was reduced. These results imply that the DNA binding activity of SRY is required for sex determination.
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            The age of occurrence of gonadal tumors in intersex patients with a Y chromosome.

            A total of 320 intersex patients with a Y chromosome were classified into four groups; (1) gonadal dysgenesis, (2) asymmetrical gonadal differentiation, (3) virilizing male hermaphroditism and (4) feminizing male hermaphroditism (testicular feminization syndrome). Of these 320 cases, 98 were from the files of The Johns Hopkins Hospital and the remainder from the literature. The incidence of tumors in relation to age and clinical classification was analyzed by computer. The results were plotted for each group. It was found that the percentage of tumors rose appreciably soon after the age of puberty in the first three groups, and it was concluded that the gonads were best removed before the age of puberty. In the case of testicular feminization patients, procrastination until the age of 25 could be considered, if one were willing to assume the risk of neoplasia of about 3.6 per cent until then.
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              A cluster of male pseudohermaphrodites with 5α-reductase deficiency in Papua New Guinea

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                Author and book information

                Book
                978-1-4757-3891-9
                978-1-4757-3889-6
                1994
                10.1007/978-1-4757-3889-6
                Book Chapter
                1994
                : 3-29
                10.1007/978-1-4757-3889-6_1

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