Puberty without gonadotropins — A novel mechanism of sexual development

Wierman, Margaret E.; Beardsworth, David; Badger, T. M.; Mansfield, Joan; Crawford, John D.; Crigler, John F.; Bode, Hans H.; Scully, Robert E.; Hoffman, William H.; Crowley, William F.

doi:10.1016/0022-4731(84)90507-7

Cited by 20 publications

(29 citation statements)

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“…The autonomous hypersecretion of testosterone could not be explained by increased gonadotropins since no immunoreactivity of these hormones could be identified in the patients' serum (178,181,182), although some evidence was presented of a factor in the serum of these boys that could stimulate monkey Leydig cells (183). Two groups demonstrated that these familial cases of LH-independent precocious puberty in boys were caused by mutations resulting in single amino acid changes in the LH receptor protein (175,176).…”

Section: Themmen and Huhtaniemimentioning

confidence: 99%

Mutations of Gonadotropins and Gonadotropin Receptors: Elucidating the Physiology and Pathophysiology of Pituitary-Gonadal Function

2000

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The recent unraveling of structures of genes for the gonadotropin subunits and gonadotropin receptors has provided reproductive endocrinologists with new tools to study normal and pathological functions of the hypothalamic-pituitary-gonadal axis. Rare inactivating mutations that produce distinctive phenotypes of isolated LH or FSH deficiency have been discovered in gonadotropin subunit genes. In addition, there is a common polymorphism in the LH␤ subunit gene with possible clinical significance as a contributing factor to pathologies of LH-dependent gonadal functions. Both activating and inactivating mutations have been detected in the gonadotropin receptor genes, a larger number in the LH receptor gene, but so far only a few in the gene for the FSH receptor. These mutations corroborate and extend our knowledge of clinical consequences of gonadotropin resistance and inappropriate gonadotropin action. The information obtained from human mutations has been complemented by animal models with disrupted or inappropriately activated gonadotropin ligand or receptor genes. These clinical and experimental genetic disease models form a powerful tool for exploring the physiology and pathophysiology of gonadotropin function and provide an excellent example of the power of molecular biological approaches in the study of pathogenesis of diseases. (Endocrine Reviews 21: 2000)

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Section: Themmen and Huhtaniemimentioning

confidence: 99%

Mutations of Gonadotropins and Gonadotropin Receptors: Elucidating the Physiology and Pathophysiology of Pituitary-Gonadal Function

2000

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“…LH stimulates gonadal ste roid release and in concert with follicle-stim ulating hormone is essential for the onset of reproductive function in all mammals stud ied to date. Rare instances in humans where puberty occurs in the absence of detectable gonadotropin activity are considered patho logical and are the subject of intense investi gation [1], Studies of the reproductive endocrinol ogy of strepsirhine primates are few, and we are aware of only one that deals with gonad otropin control in any Malagasy lemur [2], In that study, Norman et al reported that in captive Lemur catta LH-like activity was de tectable under physiological conditions, us ing the ovine-antiovine radioimmunoassay (R1A) of Niswender et al [3], This heterolo gous immunoassay for LH has been applied successfully to a wide range of mammalian taxa. Here we report the results of a series of studies on seasonal breeding in captive Mal agasy lemurs, carried out on individuals be longing to two subspecies of L. fulvus (L. fulvus rufus and L. fulvus albifrons) and to the monotypic species L. catta.…”

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Lemur Species Show Differences in Immunological and Biological Activity of Luteinizing Hormone

Dunaif¹,

Tattersall²

1991

IJFP

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“…Hall and Warrick (26) proposed the etiology was related to abnormal or excessive signals from the hypothalamus. However, the majority of patients with McCune-Albright syndrome and sexual precocity have very low levels of gonadotropins (3,4,6) and do not exhibit further suppression of gonadotropins nor cessation of menses when treated with long-acting agonists of LHRH. Thus, excess neurotransmitters or excess LHRH secretion cannot account for sexual precocity in most girls with McCune-Albright syndrome, and other etiologies must be investigated.…”

Section: Ovarian Volumes In Mccune-albright Syndrome and In Central Pmentioning

confidence: 99%

“…Advancement and regression of secondary sexual characteristics and episodes of menses in these girls often follow a cyclical course (3,4) and in most girls, are independent of pubertal secretion of gonadotropins (3)(4)(5)(6). In fact, gonadotropin levels are frequently suppressed below prepubertal levels (5).…”

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confidence: 99%

Ovarian Function in Girls with McCune-Albright Syndrome

et al. 1986

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ABSTRACT. We measured plasma estradiol levels and ovarian volumes in eight girls with precocious puberty due to McCune-Albright syndrome. Six girls had gonadotropinindependent ovarian estrogen secretion and two girls had pubertal gonadotropin levels. Mean ovarian volume in all patients was significantly greater than in normal prepubertal girls. Mean ovarian volumes of the girls with McCuneAlbright syndrome overlapped the range found in girls with idiopathic central precocious puberty or central precocious puberty associated with central nervous system lesions. However, the degree of asymmetry between the right and left ovaries was significantly greater in girls with McCuneAlbright syndrome. Asymmetry was due, for the most part, to the presence of large solitary cysts in the larger of the two ovaries. In the six girls with McCune-Albright syndrome and gonadotropin-independent precocious puberty, both mean ovarian volume and the degree of asymmetry between the right and left ovaries were significantly correlated with plasma estradiol. Serum follicle-stimulating hormone bioactivity was increased in two patients but did not vary with ovarian cyst size. Thyroid-stimulating hormone levels were normal but serum prolactin was slightly elevated in one of the six girls with gonadotropin-independent precocious puberty. Fluctuation in the size of unilateral ovarian cysts appears to result in changes in the plasma estradiol level, leading to advancement and spontaneous regression of secondary sexual characteristics and menses in girls with McCune-Albright syndrome. The cause of the cyst formation is unknown but may be related to periodic elevation of as yet undefined serum factors such as follicle-stimulating hormone bioactive substances. (Pediatr Res 20: [859][860][861][862][863] 1986) Abbreviations FSH, follicle-stimulating hormone TSH, thyroid-stimulating hormone LH, luteinizing hormone LHRH, luteinizing hormone-releasing hormone CNS, central nervous system hCG, human chorionic gonadotropin

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Puberty without gonadotropins — A novel mechanism of sexual development

Cited by 20 publications

References 0 publications

Mutations of Gonadotropins and Gonadotropin Receptors: Elucidating the Physiology and Pathophysiology of Pituitary-Gonadal Function

Mutations of Gonadotropins and Gonadotropin Receptors: Elucidating the Physiology and Pathophysiology of Pituitary-Gonadal Function

Lemur Species Show Differences in Immunological and Biological Activity of Luteinizing Hormone

Ovarian Function in Girls with McCune-Albright Syndrome

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