NDLI: Mutant Huntingtin and Elusive Defects in Oxidative Metabolism and Mitochondrial Calcium Handling

Content Provider	Springer Nature Link
Author	Brustovetsky, Nickolay
Copyright Year	2015
Abstract	Elongation of a polyglutamine (polyQ) stretch in huntingtin protein (Htt) is linked to Huntington’s disease (HD) pathogenesis. The mutation in Htt correlates with neuronal dysfunction in the striatum and cerebral cortex and eventually leads to neuronal cell death. The exact mechanisms of the injurious effect of mutant Htt (mHtt) on neurons are not completely understood but might include aberrant gene transcription, defective autophagy, abnormal mitochondrial biogenesis, anomalous mitochondrial dynamics, and trafficking. In addition, deficiency in oxidative metabolism and defects in mitochondrial Ca$^{2+}$ handling are considered essential contributing factors to neuronal dysfunction in HD and, consequently, in HD pathogenesis. Since the discovery of the mutation in Htt, the questions whether mHtt affects oxidative metabolism and mitochondrial Ca$^{2+}$ handling and, if it does, what mechanisms could be involved were in focus of numerous investigations. However, despite significant research efforts, the detrimental effect of mHtt and the mechanisms by which mHtt might impair oxidative metabolism and mitochondrial Ca$^{2+}$ handling remain elusive. In this paper, I will briefly review studies aimed at clarifying the consequences of mHtt interaction with mitochondria and discuss experimental results supporting or arguing against the mHtt effects on oxidative metabolism and mitochondrial Ca$^{2+}$ handling.
Starting Page	2944
Ending Page	2953
Page Count	10
File Format	PDF
ISSN	08937648
Journal	Molecular Neurobiology
Volume Number	53
Issue Number	5
e-ISSN	15591182
Language	English
Publisher	Springer US
Publisher Date	2015-05-05
Publisher Place	New York
Access Restriction	One Nation One Subscription (ONOS)
Subject Keyword	Mutant huntingtin Mitochondria Respiration Calcium uptake Permeability transition pore Neurosciences Neurobiology Cell Biology Neurology
Content Type	Text
Resource Type	Article
Subject	Neurology Cellular and Molecular Neuroscience

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