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Gene Review

HHG  -  hypergonadotropic hypogonadism

Homo sapiens

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Disease relevance of HHG


Psychiatry related information on HHG


High impact information on HHG


Chemical compound and disease context of HHG


Biological context of HHG

  • We have identified a missense mutation (N440I) in three patients with AHC and HHG, all belonging to a large Greenlandic family [16].
  • OBJECTIVE: Mutations of the DAX1 gene (Dosage-sensitive sex reversal-Adrenal hypoplasia congenita critical region on the X chromosome gene 1), which encodes a novel orphan nuclear receptor, have been identified in patients with X-linked adrenal hypoplasia congenita (AHC) and hypogonadotrophic hypogonadism (HHG) [17].
  • However, there was no effect of estradiol replacement on mean ovarian volume, the number or size of new follicles, or the ovulation rate in all subjects or in the subset with no identified cause for their hypergonadotropic hypogonadism (n = 20) [18].
  • Other less frequent features included neurological and psychiatric abnormalities, urodynamic abnormalities, limited joint motility, cardiovascular and gastrointestinal autonomic neuropathy, hypergonadotropic hypogonadism in males, and diabetic microvascular disease [19].
  • Aromatase deficiency in a female who is compound heterozygote for two new point mutations in the P450arom gene: impact of estrogens on hypergonadotropic hypogonadism, multicystic ovaries, and bone densitometry in childhood [20].

Anatomical context of HHG


Associations of HHG with chemical compounds


Other interactions of HHG


Analytical, diagnostic and therapeutic context of HHG


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  3. A novel mutation in DAX1 causes delayed-onset adrenal insufficiency and incomplete hypogonadotropic hypogonadism. Tabarin, A., Achermann, J.C., Recan, D., Bex, V., Bertagna, X., Christin-Maitre, S., Ito, M., Jameson, J.L., Bouchard, P. J. Clin. Invest. (2000) [Pubmed]
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  6. Clinical use of androgenic steroids at a major medical center: who prescribes androgens and how well do they do it? Cavanah, S.F., Jacobson, J.M., McIntyre, T. Journal of clinical pharmacology. (1993) [Pubmed]
  7. Clinical features of galactokinase deficiency: a review of the literature. Bosch, A.M., Bakker, H.D., van Gennip, A.H., van Kempen, J.V., Wanders, R.J., Wijburg, F.A. J. Inherit. Metab. Dis. (2002) [Pubmed]
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  9. Hypergonadotropic hypogonadism in female patients with galactosemia. Kaufman, F.R., Kogut, M.D., Donnell, G.N., Goebelsmann, U., March, C., Koch, R. N. Engl. J. Med. (1981) [Pubmed]
  10. Autoimmune adrenal insufficiency and autoimmune polyendocrine syndromes: autoantibodies, autoantigens, and their applicability in diagnosis and disease prediction. Betterle, C., Dal Pra, C., Mantero, F., Zanchetta, R. Endocr. Rev. (2002) [Pubmed]
  11. Reversal of the hypogonadotropic hypogonadism of obese men by administration of the aromatase inhibitor testolactone. Zumoff, B., Miller, L.K., Strain, G.W. Metab. Clin. Exp. (2003) [Pubmed]
  12. Hormonal abnormalities in obesity. Zumoff, B. Acta Med. Scand. Suppl. (1988) [Pubmed]
  13. Inherited disorders of glycoprotein synthesis: cell biological insights. McDowell, G., Gahl, W.A. Proc. Soc. Exp. Biol. Med. (1997) [Pubmed]
  14. The endocrine system in treated patients with classical galactosemia. Rubio-Gozalbo, M.E., Panis, B., Zimmermann, L.J., Spaapen, L.J., Menheere, P.P. Mol. Genet. Metab. (2006) [Pubmed]
  15. Substitution with testosterone as aromatizable substrate for induction of follicular maturation, estradiol production and ovulation in a patient with 17 alpha-hydroxylase deficiency. Neuwinger, J., Licht, P., Munzer, B., Sir-Petermann, T., Siebzehnrübl, E., Wildt, L. Exp. Clin. Endocrinol. Diabetes (1996) [Pubmed]
  16. X-linked adrenal hypoplasia in a large Greenlandic family. Detection of a missense mutation (N4401) in the DAX-1 gene; implication for genetic counselling and carrier diagnosis. Schwartz, M., Blichfeldt, S., Müller, J. Hum. Genet. (1997) [Pubmed]
  17. Novel DAX1 mutations in X-linked adrenal hypoplasia congenita and hypogonadotrophic hypogonadism. Bassett, J.H., O'Halloran, D.J., Williams, G.R., Beardwell, C.G., Shalet, S.M., Thakker, R.V. Clin. Endocrinol. (Oxf) (1999) [Pubmed]
  18. A randomized, controlled trial of estradiol replacement therapy in women with hypergonadotropic amenorrhea. Taylor, A.E., Adams, J.M., Mulder, J.E., Martin, K.A., Sluss, P.M., Crowley, W.F. J. Clin. Endocrinol. Metab. (1996) [Pubmed]
  19. Diabetes mellitus and optic atrophy: a study of Wolfram syndrome in the Lebanese population. Medlej, R., Wasson, J., Baz, P., Azar, S., Salti, I., Loiselet, J., Permutt, A., Halaby, G. J. Clin. Endocrinol. Metab. (2004) [Pubmed]
  20. Aromatase deficiency in a female who is compound heterozygote for two new point mutations in the P450arom gene: impact of estrogens on hypergonadotropic hypogonadism, multicystic ovaries, and bone densitometry in childhood. Mullis, P.E., Yoshimura, N., Kuhlmann, B., Lippuner, K., Jaeger, P., Harada, H. J. Clin. Endocrinol. Metab. (1997) [Pubmed]
  21. A duplication of distal Xp associated with hypogonadotrophic hypogonadism, hypoplastic external genitalia, mental retardation, and multiple congenital abnormalities. Telvi, L., Ion, A., Carel, J.C., Desguerre, I., Piraud, M., Boutin, A.M., Feingold, J., Ponsot, G., Fellous, M., McElreavey, K. J. Med. Genet. (1996) [Pubmed]
  22. Decrease of cellular ATP by dihexanoylglycerol may limit responses to protein kinase C activation. Dawson, J., Thompson, N.T., Bonser, R.W., Hodson, H.F., Garland, L.G. FEBS Lett. (1987) [Pubmed]
  23. Partial reversal of the hypogonadotropic hypogonadism of obese men by administration of corticosuppressive doses of dexamethasone. Zumoff, B., Strain, G.W., Miller, L.K., Rosner, W., Levit, C.D., Miller, E.H., Rosenfeld, R.S. International journal of obesity. (1988) [Pubmed]
  24. Increased incidence of thyroid disease among men with hypergonadotropic hypogonadism. Wortsman, J., Moses, H.W., Dufau, M.L. Am. J. Med. (1986) [Pubmed]
  25. An inactivating mutation of the luteinizing hormone receptor causes amenorrhea in a 46,XX female. Toledo, S.P., Brunner, H.G., Kraaij, R., Post, M., Dahia, P.L., Hayashida, C.Y., Kremer H Themmen, A.P. J. Clin. Endocrinol. Metab. (1996) [Pubmed]
  26. Intramuscular testosterone esters and plasma lipids in hypogonadal men: a meta-analysis. Whitsel, E.A., Boyko, E.J., Matsumoto, A.M., Anawalt, B.D., Siscovick, D.S. Am. J. Med. (2001) [Pubmed]
  27. Gonadotropin dynamics in XX males. Pérez-Palacios, G., Medina, M., Ullao-Aguirre, A., Chávez, B.A., Villareal, G., Dutrem, M.T., Cahill, L.T., Wachtel, S. J. Clin. Endocrinol. Metab. (1981) [Pubmed]
  28. Ichthyosis vulgaris with hypogenitalism and hypogonadism: evidence for different genotypes by lipoprotein electrophoresis and steroid sulfatase testing. Traupe, H., Müller-Migl, C.R., Kolde, G., Happle, R., Kövary, P.M., Hameister, H., Ropers, H.H. Clin. Genet. (1984) [Pubmed]
  29. The effect of leuprolide acetate in aiding induction of ovulation in hypergonadotropic hypogonadism: a case report. Check, J.H., Wu, C.H., Check, M.L. Fertil. Steril. (1988) [Pubmed]
  30. Novel mutation of the DAX1 gene in a patient with X-linked adrenal hypoplasia congenita and hypogonadotropic hypogonadism. Hamaguchi, K., Arikawa, M., Yasunaga, S., Kakuma, T., Fukagawa, K., Yanase, T., Nawata, H., Sakata, T. Am. J. Med. Genet. (1998) [Pubmed]
  31. Mutations of LH and FSH receptors. Beck-Peccoz, P., Romoli, R., Persani, L. J. Endocrinol. Invest. (2000) [Pubmed]
  32. Hypergonadotropic hypogonadism, SHBG deficiency and hyperprolactinaemia: a transient phenomenon during induction chemotherapy in leukemic children. Beck, W., Schwarz, S., Heidemann, P.H., Jentsch, E., Stubbe, P., König, A. Eur. J. Pediatr. (1982) [Pubmed]
  33. Endocrine late effects from multi-modality treatment of neuroblastoma. van Santen, H.M., de Kraker, J., Vulsma, T. Eur. J. Cancer (2005) [Pubmed]
  34. Clomiphene citrate therapy for male infertility. Allag, I.S., Alexander, N.J. Urology (1979) [Pubmed]
  35. Gonadal karyotyping in women with and without ovarian activity. Kemmann, E., Gentile, G., Douglas, A., Jones, J.R. Am. J. Obstet. Gynecol. (1977) [Pubmed]
  36. Normal growth and normalization of hypergonadotropic hypogonadism in atypical Turner syndrome (45,X/46,XX/47,XXX). Correlation of body height with distribution of cell lines. Partsch, C.J., Pankau, R., Sippell, W.G., Tolksdorf, M. Eur. J. Pediatr. (1994) [Pubmed]
  37. Assessment of sex chromosome composition using fluorescent in situ hybridization as an adjunct to GTG-banding. Mark, H.F., Meyers-Seifer, C.H., Seifer, D.B., Demoranville, B.M., Jackson, I.M. Ann. Clin. Lab. Sci. (1995) [Pubmed]
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