Makorin ring finger protein 3 (MKRN3)

Makorin ring finger protein 3 (MKRN3) is an imprinted, E3 ubiquitin ligase protein encoded at chromosome 15q11-q13. It contains multiple zinc finger motifs and a RING finger motif, essential for its protein-degrading enzymatic activity. MKRN3 acts as a key repressor of the onset of puberty by inhibiting neuronal secretion of GnRH from the hypothalamus. Loss-of-function mutations in the paternal MKRN3 allele are the most frequent monogenic cause of central precocious puberty. MKRN3 interacts with numerous protein partners, modulating various cellular processes including RNA metabolism and cell–cell adhesion, but its principal physiological role involves the precise timing of puberty and possibly post-transcriptional control of related gene expression.

Other names

ZNF127D15S9Makorin ring finger protein 3 (MKRN3)

Ubiquitin ligase activity (E3) attaching ubiquitin to proteins involved in GnRH regulation, proteasome-mediated degradation, transcriptional repression through promoter inhibition