Fundamentals of CNS energy metabolism and alterations in lysosomal storage diseases
JournalJournal of Neurochemistry
PublisherBlackwell Publishing Ltd
MetadataShow full item record
AbstractThe brain has a very high requirement for energy. Adult brain relies on glucose as an energy substrate, whereas developing brain can utilize alternative substrates as well as glucose for energy and for the biosynthesis of lipids and proteins required for brain development. Metabolism provides the energy required to support all cellular functions and brain development and building blocks for macromolecules. Lysosomes are organelles involved in breakdown of biological compounds including proteins and complex lipids in the body and brain. Recent studies suggest that lysosomal dysfunction can damage neurons and/or alter neurotransmitter homeostasis. Several studies also implicate mitochondrial dysfunction in the pathophysiology of brain damage in lysosomal storage diseases. This manuscript provides a brief review of energy metabolism and the key pathways involved in metabolism in brain. Roles of lysosomes related to metabolism and neurotransmission are discussed, and evidence for mitochondrial dysfunction in several lysosomal storage diseases is presented. (Figure presented.). © 2018 International Society for Neurochemistry
SponsorsThe authors gratefully acknowledge and thank Bruna Klippel Ferreira for her excellent effort in preparing Figs 1 and 2. Dr. Schuck’s research is supported by the National Council for Scientific and Technological Development (CNPq). Dr. Ferreira’s research is supported by the ‘Fundacao de Amparo a Pesquisa do Estado do Rio de Janeiro’, Coordenacao de Aperfeicoamento de Pessoal de Nível Superior–Brasil (CAPES)–Finance Code 001 and National Council for Scientific and Technological Development–Brasil (CNPq). The authors have no conflict of interest to declare. Mary C. McKenna is an editor for the Journal of Neurochemistry
Lysosomal Storage Diseases
Identifier to cite or link to this itemhttps://www.scopus.com/inward/record.uri?eid=2-s2.0-85056486468&doi=10.1111%2fjnc.14577&partnerID=40&md5=df47b6e719983e6335d65e7c2bcc3e8e; http://hdl.handle.net/10713/8545
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