Neuroendocrine complications of central nervous system malformations

Abstract

Publisher Summary The nervous system regulates the function of both endocrine and exocrine glands, not only through neurosecretion but also through the autonomic nervous system via cholinergic and noradrenergic pathways. In mice, anterior pituitary development takes place in four distinct stages: pituitary placode formation, the development of a rudimentary Rathke's pouch, the formation of a definitive pouch, and finally the terminal differentiation of various cell types in a temporally and spatially regulated manner. Several signaling molecules and transcription factors that are expressed in the neural ectoderm and not in Rathke's pouch—FGF8, BMP4, and NKX2.1—are thought to play a significant part in normal anterior pituitary development. These signaling molecules can then activate or repress key regulatory genes encoding transcription factors, such as HESX1, LHX3, and LHX4, within the developing Rathke's pouch that are essential for subsequent development of the pituitary. Disorders involving growth hormone (GH) and one or more additional pituitary hormones are caused by mutations in the homeodomain transcription factors that direct embryological development of the anterior pituitary gland.