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Maternal meiosis II nondisjunction in a case of 47,XXY testicular feminization

✍ Scribed by Ulrich Müller; Nancy R. Schneider; James F. Marks; Kenneth G. Kupke; Golder N. Wilson


Book ID
104660664
Publisher
Springer
Year
1990
Tongue
English
Weight
469 KB
Volume
84
Category
Article
ISSN
0340-6717

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✦ Synopsis


An 11-year-old patient with incomplete testicular feminization and a 47,XXY karyotype is described. The patient had female external genitalia, clitoromegaly, and some features of Klinefelter's syndrome, including speech delay and delayed intellectual development. DNA analysis using X chromosomal DNA sequences suggests that the supernumerary X chromosome in the patient resulted from maternal nondisjunction during meiosis II. The M II error thereby provides the basis for homozygosity of a mutation in the androgen receptor locus.