ArticleJournal of lipid research2012
Raft-like microdomains play a key role in mitochondrial impairment in lymphoid cells from patients with Huntington's disease.
Article in Journal of lipid research, 2012. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 18 papers.
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Who cites it
18 citing papers in PubMed, 26 citations in OpenAlex.
- Review
- Extracellular Vesicles in the Crosstalk of Autophagy and Apoptosis: A Role for Lipid Rafts.Cells · 2025Review
- Fasting the mitochondria to prevent neurodegeneration: the role of ceramides.Frontiers in neuroscience · 2025Review
- Review
- The Janus-faced functions of Apolipoproteins L in membrane dynamics.Cellular and molecular life sciences : CMLS · 2024Article
- Apolipoproteins L1 and L3 control mitochondrial membrane dynamics.Cell reports · 2023Article
- Article
- The long and the short of Huntington's disease: how the sphingolipid profile is shifted in the caudate of advanced clinical cases.Brain communications · 2022Article
- Article
- Targeting Mitochondrial Network Disorganization is Protective inAging and disease · 2021Article
- Review
- Protein Aggregation Landscape in Neurodegenerative Diseases: Clinical Relevance and Future Applications.International journal of molecular sciences · 2021Review
- Lipids in the cell: organisation regulates function.Cellular and molecular life sciences : CMLS · 2018Review
- The role of sphingolipids in psychoactive drug use and addiction.Journal of neural transmission (Vienna, Austria : 1996) · 2018Review
- Distinct membrane properties are differentially influenced by cardiolipin content and acyl chain composition in biomimetic membranes.Biochimica et biophysica acta. Biomembranes · 2017Article
- Mitochondrial lipids in neurodegeneration.Cell and tissue research · 2017Review
- Mitochondrial membrane fluidity is consistently increased in different models of Huntington disease: restorative effects of olesoxime.Molecular neurobiology · 2014Article
- Evidence for the involvement of GD3 ganglioside in autophagosome formation and maturation.Autophagy · 2014Article
Corrections and comments
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Authors and funding
11 authors at 2 institutions in 1 country.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Huntington's disease (HD) is a genetic neurodegenerative disease characterized by an exceedingly high number of contiguous glutamine residues in the translated protein, huntingtin (Htt). The primary site of cell toxicity is the nucleus, but mitochondria have been identified as key components of cell damage. The present work has been carried out in immortalized lymphocytes from patients with HD. These cells, in comparison with lymphoid cells from healthy subjects, displayed: i) a redistribution of mitochondria, forming large aggregates; ii) a constitutive hyperpolarization of mitochondrial membrane; and iii) a constitutive alteration of mitochondrial fission machinery, with high apoptotic susceptibility. Moreover, mitochondrial fission molecules, e.g., protein dynamin-related protein 1, as well as Htt, associated with mitochondrial raft-like microdomains, glycosphingolipid-enriched structures detectable in mitochondria. These findings, together with the observation that a ceramide synthase inhibitor and a raft disruptor are capable of impairing the peculiar mitochondrial remodeling in HD cells, suggest that mitochondrial alterations occurring in these cells could be due to raft-mediated defects of mitochondrial fission/fusion machinery.
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Registered trials
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