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Previous Volume 350:367-378 January 22, 2004 Number 4 Next

	Since this article has no abstract, we have provided an extract of the first 100 words of the full text and any section headings.

	Full Text PDF PDA Full Text Purchase this article Perspective by Federman, D. D. Letters Add to Personal Archive Add to Citation Manager Notify a Friend E-mail When Cited E-mail When Letters Appear Related Article PubMed Citation

Sex determination, which depends on the sex-chromosome complement of the embryo, is established by multiple molecular events that direct the development of germ cells, their migration to the urogenital ridge, and the formation of either a testis, in the presence of the Y chromosome (46,XY), or an ovary in the absence of the Y chromosome and the presence of a second X chromosome (46,XX). Sex determination sets the stage for sex differentiation, the sex-specific response of tissues to hormones produced by the gonads after they have differentiated in a male or female pattern. A number of genes have been discovered . . . [Full Text of this Article]

Germ Cells

Male Germ Cells

Female Germ Cells

Syndromes of Absent Germ Cells and Relation of Germ Cells to Stem Cells

Syndromes of Gonadal Dysgenesis

Gonadal and Renal Abnormalities

            The Frasier Syndrome and WT1

            The Denys–Drash Syndrome and WT1

Gonadal and Adrenal Abnormalities

            Steroidogenic Factor

            DAX1

            A Potential Role for DAX1 in Mixed Gonadal Dysgenesis

SRY and SRY Homeobox Genes in Pure Gonadal Dysgenesis

True Hermaphroditism

Müllerian Agenesis

Functional Abnormalities in Sexual Development

Persistent Müllerian Duct Syndrome

Male Pseudohermaphroditism

Congenital Adrenal Hyperplasia

Summary

Source Information

From the Pediatric Surgical Research Laboratories and the Pediatric Surgical Services, Massachusetts General Hospital and Harvard Medical School, Boston.

Address reprint requests to Dr. MacLaughlin or Dr. Donahoe at the Pediatric Surgical Research Laboratories, Massachusetts General Hospital, Boston, MA 02114, or at maclaughlin@helix.mgh.harvard.edu or donahoe.patricia@mgh.harvard.edu.

Related Letters:

Sex Determination, Differentiation, and Identity
Berenbaum S. A., Sandberg D. E., Baratz A. B., Migeon C. J., Berkovitz G. D., Wisniewski A. B., Tomasi P. A., Reiner W. G., Gearhart J. P., Donahoe P. K., MacLaughlin D. T.
Extract | Full Text | PDF  
N Engl J Med 2004; 350:2204-2206, May 20, 2004. Correspondence

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