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Functional mutations of the PRLR gene are related with congenital hyperprolactinemia. To determine the cause of congenital hyperprolactinemia in a patient with menstrual irregularity, we sequenced the coding region and exon-intron boundaries of the PRLR gene. A novel mutation, p.Cys746Phe (c.2195G>C), was identified in exon 14 of the PRLR gene. The mutation was predicted to result in a substitution of a hydrophobic amino acid (Phe) for a hydrophilic one (Cys), which may affect proper folding and function of the hormone receptor protein. When cotransfected with the PRLR receptor, the mutant receptor failed to stimulate the proliferation of GH4C1 cells, indicating that the p.Cys746Phe mutation may impair the function of PRLR. The mutant receptor was efficiently transported from the endoplasmic reticulum to the plasma membrane. In addition, the mutant receptor was mislocalized within the cytoplasm and might be retained in the Golgi apparatus. Taken together, these results provide functional evidence for PRLR-mediated actions of prolactin in promoting