Biometals in rare neurodegenerative disorders of childhood
- 1Department of Neurobiology, A.I. Virtanen Institute for Molecular Sciences, University of Eastern Finland, Kuopio, Finland
- 2Department of Pathology, The University of Melbourne, Parkville, VIC, Australia
Copper, iron, and zinc are just three of the main biometals critical for correct functioning of the central nervous system (CNS). They have diverse roles in many functional processes including but not limited to enzyme catalysis, protein stabilization, and energy production. The range of metal concentrations within the body is tightly regulated and when the balance is perturbed, debilitating effects ensue. Homeostasis of brain biometals is mainly controlled by various metal transporters and metal sequestering proteins. The biological roles of biometals are vastly reviewed in the literature with a large focus on the connection to neurological conditions associated with ageing. Biometals are also implicated in a variety of debilitating inherited childhood disorders, some of which arise soon following birth or as the child progresses into early adulthood. This review acts to highlight what we know about biometals in childhood neurological disorders such as Wilson's disease (WD), Menkes disease (MD), neuronal ceroid lipofuscinoses (NCLs), and neurodegeneration with brain iron accumulation (NBIA). Also discussed are some of the animal models available to determine the pathological mechanisms in these childhood disorders, which we hope will aid in our understanding of the role of biometals in disease and in attaining possible therapeutics in the future.
Keywords: metals, neurodegeneration, childhood, copper, iron, zinc
Citation: Parker SJ, Koistinaho J, White AR and Kanninen KM (2013) Biometals in rare neurodegenerative disorders of childhood. Front. Aging Neurosci. 5:14. doi: 10.3389/fnagi.2013.00014
Received: 25 January 2013; Accepted: 05 March 2013;
Published online: 25 March 2013.
Edited by:Peter Crouch, University of Melbourne, Australia
Reviewed by:Paula I. Moreira, University of Coimbra, Portugal
Junming Wang, University of Mississippi Medical Center, USA
Copyright © 2013 Parker, Koistinaho, White and Kanninen. This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits use, distribution and reproduction in other forums, provided the original authors and source are credited and subject to any copyright notices concerning any third-party graphics etc.
*Correspondence: Katja M. Kanninen, Department of Neurobiology, A.I. Virtanen Institute for Molecular Sciences, University of Eastern Finland, Yliopistonranta 1, 70211 Kuopio, Finland. e-mail: firstname.lastname@example.org