TY - JOUR T1 - MKRN3 Levels in Girls with Central Precocious Puberty during GnRHa Treatment: A Longitudinal Study. JF - Horm Res Paediatr Y1 - 2018 A1 - Grandone, Anna A1 - Cirillo, Grazia A1 - Sasso, Marcella A1 - Tornese, Gianluca A1 - Luongo, Caterina A1 - Festa, Adalgisa A1 - Marzuillo, Pierluigi A1 - Miraglia Del Giudice, Emanuele KW - Brain Diseases KW - Case-Control Studies KW - Child KW - Child, Preschool KW - Female KW - Follicle Stimulating Hormone KW - Genotype KW - Gonadotropin-Releasing Hormone KW - Humans KW - Longitudinal Studies KW - Luteinizing Hormone KW - Male KW - Puberty, Precocious KW - Ribonucleoproteins 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.

VL - 90 IS - 3 U1 - http://www.ncbi.nlm.nih.gov/pubmed/30269125?dopt=Abstract ER -