Mitochondrial Abnormalities and Synaptic Damage in Huntington’s Disease: a Focus on Defective Mitophagy and Mitochondria-Targeted Therapeutics

Huntington’s disease (HD) is a fatal and pure genetic disease with a progressive loss of medium spiny neurons (MSN). HD is caused by expanded polyglutamine repeats in the exon 1 of HD gene. Clinically, HD is characterized by chorea, seizures, involuntary movements, dystonia, cognitive decline, intel...

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Veröffentlicht in:	Molecular neurobiology 2021-12, Vol.58 (12), p.6350-6377
Hauptverfasser:	Sawant, Neha, Morton, Hallie, Kshirsagar, Sudhir, Reddy, Arubala P., Reddy, P. Hemachandra
Format:	Artikel
Sprache:	eng
Schlagworte:	Animal models Animals Axonal transport Bioenergetics Biomedical and Life Sciences Biomedicine Calcium homeostasis Cell Biology Cell culture Chorea Cognitive ability Degeneration Disease Models, Animal DNA damage Dynamin Dystonia Enzymatic activity Genetic disorders Glutamic acid receptors Humans Huntingtin Huntington Disease - metabolism Huntington Disease - pathology Huntington's disease Huntingtons disease Mitochondria Mitochondria - metabolism Mitochondria - pathology Mitochondrial DNA Mitophagy Mitophagy - physiology N-Methyl-D-aspartic acid receptors Neurobiology Neurology Neurons - metabolism Neurons - pathology Neurosciences Oxidative stress Polyglutamine Protein interaction Protein transport Proteins Seizures Spiny neurons Synapses - metabolism Synapses - pathology Trinucleotide repeat diseases
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