Huntington's disease (HD) is a progressive, fatal neurodegenerative disease caused by expanded polyglutamine repeats in the HD gene. HD is characterized by chorea, seizures, involuntary movements, dystonia, cognitive decline, intellectual impairment and emotional disturbances. Research into mutant huntingtin (Htt) and mitochondria has found that mutant Htt interacts with the mitochondrial protein dynamin-related protein 1 (Drp1), enhances GTPase Drp1 enzymatic activity, and causes excessive mitochondrial fragmentation and abnormal distribution, leading to defective axonal transport of mitochondria and selective synaptic degeneration. This article summarizes latest developments in HD research and focuses on the role of abnormal mitochondrial dynamics and defective axonal transport in HD neurons. This article also discusses the therapeutic strategies that decrease mitochondrial fragmentation and neuronal damage in HD.
Download full-text PDF |
Source |
|---|---|
| http://www.ncbi.nlm.nih.gov/pmc/articles/PMC3249480 | PMC |
| http://dx.doi.org/10.1016/j.bbadis.2011.10.016 | DOI Listing |
Publication Analysis
Top Keywords
Similar Publications
Magnetic field-oriented conductive decellularized extracellular matrix hydrogel synergizes with electrical stimulation to promote spinal cord injury repair and electrophysiological function restoration.
Biomater Adv
December 2024
Key Laboratory for Ultrafine Materials of Ministry of Education, Frontiers Science Center for Materiobiology and Dynamic Chemistry, Engineering Research Center of Biomedical Materials of Ministry of Education, School of Materials Science and Engineering, East China University of Science and Technology, Shanghai 200237, PR China. Electronic address:
Spinal cord injury (SCI) results in electrophysiological and behavioral dysfunction. Electrical stimulation (ES) is considered to be an effective treatment for mild SCI; however, ES is not applicable to severe SCI due to the disruption of electrical conduction caused by tissue defects. Therefore, the use of conductive materials to fill the defects and restore electrical conduction in the spinal cord is a promising therapeutic strategy.
View Article and Find Full Text PDFThe role of RGC degeneration in the pathogenesis of glaucoma.
Int J Biol Sci
January 2025
Department of Ophthalmology, The Second Hospital of Jilin University, Jilin University, Changchun 130000, Jilin, China.
Glaucoma is a neurodegenerative disorder marked by the loss of retinal ganglion cells (RGCs) and axonal degeneration, resulting in irreversible vision impairment. While intraocular pressure (IOP) is presently acknowledged as the sole modifiable risk factor, the sensitivity of RGCs to IOP varies among individuals. Consequently, progressive vision loss may ensue even when IOP is effectively managed.
View Article and Find Full Text PDFOlig2 single-colony-derived cranial bone-marrow mesenchymal stem cells achieve improved regeneration in a cuprizone-induced demyelination mouse model.
J Zhejiang Univ Sci B
September 2024
Center for Rehabilitation Medicine, Department of Neurosurgery, Zhejiang Provincial People's Hospital (Affiliated People's Hospital, Hangzhou Medical College), Hangzhou 310014, China.
Article Synopsis
- Oligodendrocytes, the myelinating cells in the central nervous system, are often damaged during brain injuries and neurodegenerative diseases, leading to conditions like multiple sclerosis (MS), which is characterized by myelin loss and neurological deficits.
- Current MS treatments mainly focus on reducing relapses in the early stages but do not cure the disease or help during its progressive phase, especially in more severe cases.
- Mesenchymal stem cells (MSCs) show promise for regenerative medicine due to their ability to differentiate into neural cells and their low risk for complications, with recent studies exploring their effectiveness in treating demyelination in a mouse model.
The effects of secretome of umbilical cord mesenchymal stem cells on regeneration of sciatic nerve defects in Sprague dawley rats.
PLoS One
December 2024
Faculty of Medicine Universitas Indonesia, Department of Orthopaedics & Traumatology, Dr Cipto Mangunkusumo National Central Hospital, Jakarta, Indonesia.
Background And Purpose: Current treatments for peripheral nerve defects are suboptimal. Mesenchymal stem cell (MSC) implantation holds promise, with studies indicating their efficacy through the secretome. This study aims to assess the secretome's potency in regenerating peripheral nerve defects.
View Article and Find Full Text PDFAn unstable variant of GAP43 leads to neurodevelopmental deficiency.
Sci Rep
December 2024
Department of Molecular Neurobiology, Institute for Developmental Research, Aichi Developmental Disability Center, 713-8 Kamiya, Kasugai, Aichi, 480-0392, Japan.
Growth-associated protein 43 (GAP43) is a membrane-associated phosphoprotein predominantly expressed in the nervous systems, and controls axonal growth, branching, and pathfinding. While the association between GAP43 and human neurological disorders have been reported, the underlying mechanisms remain largely unknown. We performed whole exome sequencing on a patient with intellectual disability (ID), neurodevelopmental disorders, short stature, and skeletal abnormalities such as left-right difference in legs and digital deformities, and identified a heterozygous missense variation in the GAP43 gene [NM_001130064.
View Article and Find Full Text PDFWant AI Summaries of new PubMed Abstracts delivered to your In-box?
Enter search terms and have AI summaries delivered each week - change queries or unsubscribe any time!