Abstract
The hypothalamus is a heterogeneous rostral forebrain region that regulates physiological processes essential for survival, energy metabolism, and reproduction, mainly mediated by the pituitary gland. In the updated prosomeric model, the hypothalamus represents the rostralmost forebrain, composed of two segmental regions (terminal and peduncular hypothalamus), which extend respectively into the non-evaginated preoptic telencephalon and the evaginated pallio-subpallial telencephalon. Complex genetic cascades of transcription factors and signaling molecules rule their development. Alterations of some of these molecular mechanisms acting during forebrain development are associated with more or less severe hypothalamic and pituitary dysfunctions, which may be associated with brain malformations such as holoprosencephaly or septo-optic dysplasia. Studies on transgenic mice with mutated genes encoding critical transcription factors implicated in hypothalamic-pituitary development are contributing to understanding the high clinical complexity of these pathologies. In this review article, we will analyze first the complex molecular genoarchitecture of the hypothalamus resulting from the activity of previous morphogenetic signaling centers and secondly some malformations related to alterations in genes implicated in the development of the hypothalamus.
Highlights
The hypothalamus is a highly complex brain territory held to regulate homeostasis and multiple visceral and somatic functions, many of them mediated by the pituitary gland (Saper and Lowell, 2014; Placzek et al, 2020)
The prosomeric model is uniquely consistent with the multitude of brain developmental gene expression patterns accrued during the last 40 years, which were meaningless within the columnar model
The hypothalamic ventricular organ (HVO, or paraventricular organ, HPV) is a linear longitudinal ependymal specialization running across basal proliferation. The lateral hypothalamus (PHy) and THy (Figure 6A), which is well-known in non-mammalian vertebrates and is not generally described in mammals
Summary
Edited by: Alfonso Represa, INSERM U901 Institut de Neurobiologie de la Méditerranée, France. Reviewed by: Valérie Dupé, UMR6290 Institut de Genetique et Developpement de Rennes (IGDR), France Roberta Haddad-Tóvolli, Institut de Recerca Biomèdica August Pi i Sunyer (IDIBAPS), Spain. Complex genetic cascades of transcription factors and signaling molecules rule their development. Alterations of some of these molecular mechanisms acting during forebrain development are associated with more or less severe hypothalamic and pituitary dysfunctions, which may be associated with brain malformations such as holoprosencephaly or septooptic dysplasia. Studies on transgenic mice with mutated genes encoding critical transcription factors implicated in hypothalamic-pituitary development are contributing to understanding the high clinical complexity of these pathologies. We will analyze first the complex molecular genoarchitecture of the hypothalamus resulting from the activity of previous morphogenetic signaling centers and secondly some malformations related to alterations in genes implicated in the development of the hypothalamus
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