Abstract
It is now firmly established that the mammalian brain depends on adequate expression of selenoproteins. Genetic studies in the mouse have paved the way and suggested that neurodevelopmental and neurodegenerative disorders can be caused by impaired selenoprotein expression within the brain. With technical advances in human genetics, it became clear that inborn errors of selenium (Se) metabolism or selenoprotein expression do occur and can lead to specific developmental and degenerative disoders. The discovery of spontaneous neurological phenotypes in selenoprotein P-deficient (Sepp1 −/−) mice clearly marked a turning point in our appreciation of Se and selenoproteins within the nervous system. Since then, several selenoproteins have been identified that are essential for normal brain function. Feeding animals low Se-containing diets was not sufficient to impair brain function unless the Sepp1-receptor ApoER2 was inactivated. Sepp1 and its receptors are thus pivotal in preferential Se transport into the brain. The two selenoproteins essential for mouse neurons are glutathione peroxidase 4 (Gpx4) and selenoprotein T (Selt). This chapter will focus on neurological diseases and phenotypes observed in mouse models or patients with impaired selenoprotein expression in the brain. Common observations and divergent findings will be discussed.
This is a preview of subscription content, log in via an institution to check access.
Access this chapter
Tax calculation will be finalised at checkout
Purchases are for personal use only
Similar content being viewed by others
References
E Wallace et al 1983 Gamete Res 4:377
D Behne et al 1988 Biochim Biophys Acta 966:12
G Bermano et al 1995 Biochem J 311:425
U Schweizer et al 2004 Brain Res Brain Res Rev 45:164
BA Zachara, A Pilecki 2001 Med Sci Monit 7:1002
GF Weber et al 1991 Lancet 337:1443
VT Ramaekers et al 1994 Neuropediatrics 25:217
L Schomburg et al 2003 Biochem J 370:397
KE Hill et al 2003 J Biol Chem 278:13640
MW Pitts et al 2012 Neuroscience 208:58
U Schweizer et al 2004 Biochem J 378:21
KE Hill et al 2004 J Nutr 134:157
WM Valentine et al 2005 Toxicol Pathol 33:570
U Schweizer et al 2005 Biochem J 386:221
MM Peters et al 2006 Mol Neurodegener 1:12
KE Hill et al 2007 J Biol Chem 282:10972
MW Pitts et al 2015 J Neurosci 35:15326
K Renko et al 2008 Biochem J 409:741
M Kuhbacher et al 2009 J Neurochem 110:133
M Scharpf et al 2007 J Neural Transm 114:877
KE Hill et al 2012 J Biol Chem 287:40414
GE Olson et al 2007 J Biol Chem 282:12290
RF Burk et al 2007 J Neurosci 27:6207
WM Valentine et al 2008 J Neuropathol Exp Neurol 67:68
U Schweizer 2012 in Selenium. Its Molecular Biology and Role in Human Health, DL Hatfield et al Eds (Springer Science + BusinessMedia, LLC, New York) p 235
RF Burk et al 2014 FASEB J 28:3579
GE Olson et al 2008 J Biol Chem 283:6854
J Chiu-Ugalde et al 2010 Biochem J 431:103
S Kurokawa et al 2014 Free Radic Biol Med 69:67
EK Wirth et al 2010 FASEB J 24:844
Y Zhang et al 2008 J Biol Chem 283:2427
A Seiler et al 2008 Cell Metab 8:237
S Roth et al 2010 J Trace Elem Med Biol 24:130
EK Wirth et al 2014 Biol Trace Elem Res 158:203
BA Carlson et al 2009 Biochem J 418:61
MV Kasaikina et al 2013 Carcinogenesis 34:1089
S Seeher et al 2014 Antioxid Redox Signal 21:835
S Seeher et al 2014 Biochem J 462:67
AV Raman et al 2012 Genes Brain Behav 11:601
H Imai et al 2003 Biochem Biophys Res Commun 305:278
LJ Yant et al 2003 Free Radic Biol Med 34:496
SE Yoo et al 2012 Free Radic Biol Med 52:1820
JP Friedmann Angeli et al 2014 Nat Cell Biol 16:1180
L Chen et al 2015 J Biol Chem 290:28097
M Conrad et al 2004 Mol Cell Biol 24:9414
C Jakupoglu et al 2005 Mol Cell Biol 25:1980
J Soerensen et al 2008 PLoS One 3:e1813
MJ Schneider et al 2006 Endocrinology 147:580
MJ Schneider et al 2001 Mol Endocrinol 15:2137
ME Gilbert et al 2007 Endocrinology 148:92
L Ng et al 2004 Proc Natl Acad Sci U S A 101:3474
L Ng et al 2009 Endocrinology 150:1952
L Ng et al 2010 J Neurosci 30:3347
MT Castex et al 2015 Mol Neurobiol doi:10.1007/s12035-015-9505-7
L Boukhzar et al 2016 Antioxid Redox Signal 24:557
B Ben Zeev et al 2003 J Med Genet 40:e96
O Agamy et al 2010 Am J Hum Genet 87:538
AK Anttonen et al 2015 Neurology 85:306
E Pavlidou et al 2016 Eur J Paediatr Neurol 20:483
S Palioura et al 2009 Science 325:321
K Iwama et al 2016 J Hum Genet doi:10.1038/jhg.2016.9
MR Sedaghatian 1980 Am J Med Genet 6:269
C Aygun et al 2012 Am J Med Genet A 158A:1400
AC Smith et al 2014 J Med Genet 51:470
R Prasad et al 2014 J Clin Endocrinol Metab 99:E1556
E Holzerova et al 2016 Brain 139:346
AM Dumitrescu et al 2005 Nat Genet 37:1247
E Schoenmakers et al 2010 J Clin Invest 120:4220
MF Azevedo et al 2010 J Clin Endocrinol Metab 95:4066
T Hamajima et al 2012 Eur J Endocrinol 166:757
C Di Cosmo et al 2009 J Clin Endocrinol Metab 94:4003
E Schoenmakers et al 2016 J Clin Invest 126:992
Acknowledgments
This work was supported by Deutsche Forschungsgemeinschaft (DFG), Deutscher Akademischer Austauschdienst (DAAD), and Rheinische Friedrich-Wilhelms-Universität Bonn.
Author information
Authors and Affiliations
Corresponding author
Editor information
Editors and Affiliations
Rights and permissions
Copyright information
© 2016 Springer Science+Business Media, LLC
About this chapter
Cite this chapter
Schweizer, U. (2016). Selenoproteins in Nervous System Development, Function and Degeneration. In: Hatfield, D., Schweizer, U., Tsuji, P., Gladyshev, V. (eds) Selenium. Springer, Cham. https://doi.org/10.1007/978-3-319-41283-2_36
Download citation
DOI: https://doi.org/10.1007/978-3-319-41283-2_36
Published:
Publisher Name: Springer, Cham
Print ISBN: 978-3-319-41281-8
Online ISBN: 978-3-319-41283-2
eBook Packages: Biomedical and Life SciencesBiomedical and Life Sciences (R0)