Pankov Yu A
Endocrinology Research Center, Ministry of Health of the Russian Federation, Moscow, 115778, Russia.
Mol Biol (Mosk). 2015 Sep-Oct;49(5):707-15. doi: 10.7868/S0026898415050134.
Kisspeptin (KISS1) and its receptor (KISS1R) are important regulators of the reproductive function, along with gonadoliberin (GnRH), gonadotropins (luteinizing hormone (LH) and follicle-stimulating hormone (FSH)), and sex steroid hormones. Mutations of their genes alter sexual maturation. The p.P74S, p.H90D, and p.P110T missense mutations of KISS1 are associated with central precocious puberty (CPP); and the p.G35S, p.C53R, and p.F117L mutations, with delayed puberty and isolated hypogonadotropic hypogonadism (IHH). The p.P196H and p.R386P mutations of KISS1R are also associated with CPP. However, a greater number of KISS1R mutations are associated with IHH, as is the case with p.L102P, p.L148S, p.E232Q, p.R297L, p.Y313H, pX399R, and more complex mutations, such as the 155-bp deletion that removes the acceptor splice site of intron 4 and part of exon 5, a deletion of the GCA triplet in position-2 ...-4 of intron 2, and an ACCGGCT insertion in the same site. The heterozygous compound mutations p.C223R/p.R297L and p.R331X/X399R and the 1-bp insertion 1001_1002insC of KISS1R are similarly associated with IHH. Leptin-dependent activation of KISS1 in hypothalamic neurons was observed in mice and sheep, being especially evident after puberty. Leptin exerts a permissive effect in regulating fertility and facilitate the induction of puberty by hypothalamic KISS1 and GnRH and pituitary LH and FSH, which support the reproductive function during further life.
亲吻素(KISS1)及其受体(KISS1R)是生殖功能的重要调节因子,与促性腺激素释放激素(GnRH)、促性腺激素(黄体生成素(LH)和卵泡刺激素(FSH))以及性类固醇激素一起发挥作用。它们基因的突变会改变性成熟。KISS1的p.P74S、p.H90D和p.P110T错义突变与中枢性性早熟(CPP)相关;而p.G35S、p.C53R和p.F117L突变则与青春期延迟和孤立性低促性腺激素性性腺功能减退(IHH)相关。KISS1R的p.P196H和p.R386P突变也与CPP相关。然而,更多的KISS1R突变与IHH相关,例如p.L102P、p.L148S、p.E232Q、p.R297L、p.Y313H、pX399R,以及更复杂的突变,如155bp缺失,该缺失去除了内含子4的受体剪接位点和外显子5的一部分,内含子2第 -2...-4位的GCA三联体缺失,以及同一位置的ACCGGCT插入。KISS1R的杂合复合突变p.C223R/p.R297L和p.R331X/X399R以及1bp插入1001_1002insC同样与IHH相关。在小鼠和绵羊中观察到下丘脑神经元中瘦素依赖性激活KISS1,在青春期后尤为明显。瘦素在调节生育能力方面发挥允许作用,并促进下丘脑KISS1和GnRH以及垂体LH和FSH诱导青春期,这些在后续生命中支持生殖功能。