| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH |
| |||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||||
Submitted on March 10, 2005
Accepted on June 6, 2005
Division of Endocrinology, Department of Medicine, Harbor-UCLA Medical Center and Los Angeles Biomedical Research Institute, Torrance, California 90509; Department of Psychology, Pathology and Laboratory Medicine, UCLA, California 90095
* To whom correspondence should be addressed. E-mail: Swerdloff{at}labiomed.org.
Klinefelter syndrome (XXY males) is the most common sex chromosome aneuploidy. XXY mice were generated by using a four-generation breeding scheme that involves the use of a structurally rearranged Y chromosome, Y*, yielding approximately 50% of the live born male offspring in the fourth generation with a XXY karyotype. Adult XXY mice have small testes, decreased plasma T levels, and elevated plasma FSH levels. The testes of adult XXY mice contained small seminiferous tubules with intraepithelial vacuolization and absence of germ cells whereas Leydig cells appeared to be more abundant than their XY littermates. Androgen receptor (AR) immunoexpression was localized in Leydig cells and peritubular myoid cells in both XY and XXY mice. AR immunoexpression was abundant in the Sertoli cells of XY mice but nearly absent in those of XXY mice. The testicular phenotype was marked by a 23.1% decrease in testis weights in XXY pups beginning at day 7 after birth. Gonocyte numbers were similar in XY and XXY mice at day 1 of age, followed by a 62.6% decrease in the number of gonocytes in the XXY mice on day 3 and further progressive loss in spermatogonia by days 5 and 7. On day 10, only a few spermatogonia remained in the XXY mice. To determine if the phenotype of XXY mice extended into the neurobehavioral domain, studies were conducted demonstrating impairment of learning and memory function in XXY mice. We conclude that adult XXY mice have testicular failure and learning deficits, similar to its human counterpart, Klinefelter syndrome.
This article has been cited by other articles:
 |
|
 |
|
  Y. Lue, K. Erkkila, P. Y. Liu, K. Ma, C. Wang, A. S. Hikim, and R. S. Swerdloff Fate of Bone Marrow Stem Cells Transplanted into the Testis: Potential Implication for Men with Testicular Failure Am. J. Pathol., March 1, 2007; 170(3): 899 - 908. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 A. M. Wikstrom, C. E. Hoei-Hansen, L. Dunkel, and E. Rajpert-De Meyts Immunoexpression of Androgen Receptor and Nine Markers of Maturation in the Testes of Adolescent Boys with Klinefelter Syndrome: Evidence for Degeneration of Germ Cells at the Onset of Meiosis J. Clin. Endocrinol. Metab., February 1, 2007; 92(2): 714 - 719. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 V. L.J. Tybulewicz and E. M.C. Fisher New techniques to understand chromosome dosage: mouse models of aneuploidy Hum. Mol. Genet., October 15, 2006; 15(suppl_2): R103 - R109. [Abstract] [Full Text] [PDF]
|
|
|
|
|
|
E. Itti, I. T. Gaw Gonzalo, A. Pawlikowska-Haddal, K. B. Boone, A. Mlikotic, L. Itti, F. S. Mishkin, and R. S. Swerdloff The Structural Brain Correlates of Cognitive Deficits in Adults with Klinefelter's Syndrome J. Clin. Endocrinol. Metab., April 1, 2006; 91(4): 1423 - 1427. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 J. D. Gatewood, A. Wills, S. Shetty, J. Xu, A. P. Arnold, P. S. Burgoyne, and E. F. Rissman Sex chromosome complement and gonadal sex influence aggressive and parental behaviors in mice. J. Neurosci., February 22, 2006; 26(8): 2335 - 2342. [Abstract] [Full Text] [PDF]
|
|
|
|
 |
|
 L. Aksglaede, A. M. Wikstrom, E. R.-D. Meyts, L. Dunkel, N. E. Skakkebaek, and A. Juul Natural history of seminiferous tubule degeneration in Klinefelter syndrome Hum. Reprod. Update, January 1, 2006; 12(1): 39 - 48. [Abstract] [Full Text] [PDF]
|
|
| HOME | HELP | FEEDBACK | SUBSCRIPTIONS | ARCHIVE | SEARCH |
| Endocrinology | Endocrine Reviews | J. Clin. End. & Metab. |
| Molecular Endocrinology | Recent Prog. Horm. Res. | All Endocrine Journals |
