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儿童神经发育障碍中的能量代谢。

Energy metabolism in childhood neurodevelopmental disorders.

机构信息

Neurometabolic Unit and Laboratory of Synaptic Metabolism. IPR, CIBERER (ISCIII) and MetabERN, Hospital Sant Joan de Déu, Barcelona, Spain.

Center for Scientific Studies - CECs, Valdivia 5110466, Chile.

出版信息

EBioMedicine. 2021 Jul;69:103474. doi: 10.1016/j.ebiom.2021.103474. Epub 2021 Jul 10.

Abstract

Whereas energy function in the aging brain and their related neurodegenerative diseases has been explored in some detail, there is limited knowledge about molecular mechanisms and brain networks of energy metabolism during infancy and childhood. In this review we describe current insights on physiological brain energetics at prenatal and neonatal stages, and in childhood. We then describe the main groups of inborn errors of energy metabolism affecting the brain. Of note, scarce basic neuroscience research in this field limits the opportunity for these disorders to provide paradigms of energy utilization during neurodevelopment. Finally, we report energy metabolism disturbances in well-known non-metabolic neurodevelopmental disorders. As energy metabolism is a fundamental biological function, brain energy utilization is likely altered in most neuropediatric diseases. Precise knowledge on mechanisms of brain energy disturbance will open the possibility of metabolic modulation therapies regardless of disease etiology.

摘要

尽管人们已经对衰老大脑中的能量及其相关神经退行性疾病进行了一些详细的研究,但对于婴儿期和儿童期的能量代谢的分子机制和大脑网络知之甚少。在这篇综述中,我们描述了关于产前和新生儿期以及儿童期生理大脑能量学的最新见解。然后,我们描述了影响大脑的主要组别的先天性能量代谢错误。值得注意的是,该领域缺乏基础神经科学研究限制了这些疾病作为神经发育过程中能量利用的范例的机会。最后,我们报告了在著名的非代谢性神经发育障碍中存在能量代谢紊乱。由于能量代谢是一种基本的生物学功能,因此大脑的能量利用可能在大多数神经儿科疾病中发生改变。对大脑能量紊乱机制的精确了解将为代谢调节治疗开辟可能性,而与疾病的病因无关。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/35b5/8324816/c1ecb40a7cba/gr1.jpg

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