MKRN3 Levels in Girls with Central Precocious Puberty during GnRHa Treatment: A Longitudinal Study

Anna Grandone, Grazia Cirillo, Marcella Sasso, Gianluca Tornese, Caterina Luongo, Adalgisa Festa, Pierluigi Marzuillo, Emanuele Miraglia Del Giudice

Research output: Contribution to journalArticle

Abstract

BACKGROUND: Recently, mutations of makorin RING finger protein 3 (MKRN3) have been identified in familial central precocious puberty (CPP). Serum levels of this protein decline before the pubertal onset in healthy girls and boys and are lower in patients with CPP compared to prepubertal matched pairs. The aim of our study was to investigate longitudinal changes in circulating MKRN3 levels in patients with CPP before and during GnRH analogs (GnRHa) treatment.

METHODS: We performed a longitudinal prospective study. We enrolled 15 patients with CPP aged 7.2 years (range: 2-8) with age at breast development onset < 8 years and 12 control girls matched for the time from puberty onset (mean age 11.8 ± 1.2 years). Serum values of MKRN3, gonadotropins, and 17β-estradiol were evaluated before and during treatment with GnRHa (at 6 and 12 months). The MKRN3 gene was genotyped in CPP patients. In the girls from the control group, only basal levels were analyzed.

RESULTS: No MKRN3 mutations were found among CPP patients. MKRN3 levels declined significantly from baseline to 6 months of GnRHa treatment (p = 0.0007) and from 6 to 12 months of treatment (p = 0.003); MKRN3 levels at 6 months were significantly lower than in the control girls (p < 0.0001).

CONCLUSIONS: We showed that girls with CPP had a decline in peripheral levels of MKRN3 during GnRHa treatment. Our data suggest a suppression of MKRN3 by continuous pharmacological administration of GnRHa.

Original languageEnglish
Pages (from-to)190-195
Number of pages6
JournalHormone Research in Paediatrics
Volume90
Issue number3
DOIs
Publication statusPublished - 2018

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Gonadotropin-Releasing Hormone
Longitudinal Studies
Proteins
Therapeutics
Central Precocious Puberty
Mutation
Puberty
Gonadotropins
Age of Onset
Blood Proteins
Estradiol
Breast
Prospective Studies
Pharmacology
Control Groups
Serum

Cite this

Grandone, A., Cirillo, G., Sasso, M., Tornese, G., Luongo, C., Festa, A., ... Miraglia Del Giudice, E. (2018). MKRN3 Levels in Girls with Central Precocious Puberty during GnRHa Treatment: A Longitudinal Study. Hormone Research in Paediatrics, 90(3), 190-195. https://doi.org/10.1159/000493134

MKRN3 Levels in Girls with Central Precocious Puberty during GnRHa Treatment : A Longitudinal Study. / Grandone, Anna; Cirillo, Grazia; Sasso, Marcella; Tornese, Gianluca; Luongo, Caterina; Festa, Adalgisa; Marzuillo, Pierluigi; Miraglia Del Giudice, Emanuele.

In: Hormone Research in Paediatrics, Vol. 90, No. 3, 2018, p. 190-195.

Research output: Contribution to journalArticle

Grandone, A, Cirillo, G, Sasso, M, Tornese, G, Luongo, C, Festa, A, Marzuillo, P & Miraglia Del Giudice, E 2018, 'MKRN3 Levels in Girls with Central Precocious Puberty during GnRHa Treatment: A Longitudinal Study', Hormone Research in Paediatrics, vol. 90, no. 3, pp. 190-195. https://doi.org/10.1159/000493134
Grandone, Anna ; Cirillo, Grazia ; Sasso, Marcella ; Tornese, Gianluca ; Luongo, Caterina ; Festa, Adalgisa ; Marzuillo, Pierluigi ; Miraglia Del Giudice, Emanuele. / MKRN3 Levels in Girls with Central Precocious Puberty during GnRHa Treatment : A Longitudinal Study. In: Hormone Research in Paediatrics. 2018 ; Vol. 90, No. 3. pp. 190-195.
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abstract = "BACKGROUND: Recently, mutations of makorin RING finger protein 3 (MKRN3) have been identified in familial central precocious puberty (CPP). Serum levels of this protein decline before the pubertal onset in healthy girls and boys and are lower in patients with CPP compared to prepubertal matched pairs. The aim of our study was to investigate longitudinal changes in circulating MKRN3 levels in patients with CPP before and during GnRH analogs (GnRHa) treatment.METHODS: We performed a longitudinal prospective study. We enrolled 15 patients with CPP aged 7.2 years (range: 2-8) with age at breast development onset < 8 years and 12 control girls matched for the time from puberty onset (mean age 11.8 ± 1.2 years). Serum values of MKRN3, gonadotropins, and 17β-estradiol were evaluated before and during treatment with GnRHa (at 6 and 12 months). The MKRN3 gene was genotyped in CPP patients. In the girls from the control group, only basal levels were analyzed.RESULTS: No MKRN3 mutations were found among CPP patients. MKRN3 levels declined significantly from baseline to 6 months of GnRHa treatment (p = 0.0007) and from 6 to 12 months of treatment (p = 0.003); MKRN3 levels at 6 months were significantly lower than in the control girls (p < 0.0001).CONCLUSIONS: We showed that girls with CPP had a decline in peripheral levels of MKRN3 during GnRHa treatment. Our data suggest a suppression of MKRN3 by continuous pharmacological administration of GnRHa.",
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T1 - MKRN3 Levels in Girls with Central Precocious Puberty during GnRHa Treatment

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AU - Grandone, Anna

AU - Cirillo, Grazia

AU - Sasso, Marcella

AU - Tornese, Gianluca

AU - Luongo, Caterina

AU - Festa, Adalgisa

AU - Marzuillo, Pierluigi

AU - Miraglia Del Giudice, Emanuele

N1 - © 2018 S. Karger AG, Basel.

PY - 2018

Y1 - 2018

N2 - BACKGROUND: Recently, mutations of makorin RING finger protein 3 (MKRN3) have been identified in familial central precocious puberty (CPP). Serum levels of this protein decline before the pubertal onset in healthy girls and boys and are lower in patients with CPP compared to prepubertal matched pairs. The aim of our study was to investigate longitudinal changes in circulating MKRN3 levels in patients with CPP before and during GnRH analogs (GnRHa) treatment.METHODS: We performed a longitudinal prospective study. We enrolled 15 patients with CPP aged 7.2 years (range: 2-8) with age at breast development onset < 8 years and 12 control girls matched for the time from puberty onset (mean age 11.8 ± 1.2 years). Serum values of MKRN3, gonadotropins, and 17β-estradiol were evaluated before and during treatment with GnRHa (at 6 and 12 months). The MKRN3 gene was genotyped in CPP patients. In the girls from the control group, only basal levels were analyzed.RESULTS: No MKRN3 mutations were found among CPP patients. MKRN3 levels declined significantly from baseline to 6 months of GnRHa treatment (p = 0.0007) and from 6 to 12 months of treatment (p = 0.003); MKRN3 levels at 6 months were significantly lower than in the control girls (p < 0.0001).CONCLUSIONS: We showed that girls with CPP had a decline in peripheral levels of MKRN3 during GnRHa treatment. Our data suggest a suppression of MKRN3 by continuous pharmacological administration of GnRHa.

AB - BACKGROUND: Recently, mutations of makorin RING finger protein 3 (MKRN3) have been identified in familial central precocious puberty (CPP). Serum levels of this protein decline before the pubertal onset in healthy girls and boys and are lower in patients with CPP compared to prepubertal matched pairs. The aim of our study was to investigate longitudinal changes in circulating MKRN3 levels in patients with CPP before and during GnRH analogs (GnRHa) treatment.METHODS: We performed a longitudinal prospective study. We enrolled 15 patients with CPP aged 7.2 years (range: 2-8) with age at breast development onset < 8 years and 12 control girls matched for the time from puberty onset (mean age 11.8 ± 1.2 years). Serum values of MKRN3, gonadotropins, and 17β-estradiol were evaluated before and during treatment with GnRHa (at 6 and 12 months). The MKRN3 gene was genotyped in CPP patients. In the girls from the control group, only basal levels were analyzed.RESULTS: No MKRN3 mutations were found among CPP patients. MKRN3 levels declined significantly from baseline to 6 months of GnRHa treatment (p = 0.0007) and from 6 to 12 months of treatment (p = 0.003); MKRN3 levels at 6 months were significantly lower than in the control girls (p < 0.0001).CONCLUSIONS: We showed that girls with CPP had a decline in peripheral levels of MKRN3 during GnRHa treatment. Our data suggest a suppression of MKRN3 by continuous pharmacological administration of GnRHa.

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