Lipid dysregulation in polyglutamine diseases
6 affiliations
- 1Department of Genetics, Yale School of Medicine, New Haven, CT 06510, USA
- 2Department of Molecular, Cellular, and Developmental Biology, Yale College, New Haven, CT, 06510, USA
- 3Department of Neuroscience, Yale School of Medicine, New Haven, CT 06510, USA
- 4Program in Cellular Neuroscience, Neurodegeneration and Repair, Yale School of Medicine, New Haven, CT 06510, USA
- 5Ludwig Program for the Study of Neuroimmune Interactions in Dementia, Yale School of Medicine, New Haven, CT 06510, USA
- 6Wu Tsai Institute, Yale School of Medicine, New Haven, CT 06510, USA
Abstract
Polyglutamine (polyQ) diseases are fatal neurodegenerative disorders caused by pathological cytosine-adenine-guanine (CAG) repeat expansions in disease-associated genes. They are characterized by progressive motor dysfunction and typically late onset. Although multiple mechanisms have been proposed, accumulating evidence indicates that lipid dysregulation contributes to neuronal dysfunction and degeneration. Lipids are essential for brain development and homeostasis, and disrupted lipid metabolism is implicated across neurodegenerative conditions. Within the polyQ spectrum, Huntington’s disease is the best studied and shows strong links between altered lipid pathways and neuronal pathology, whereas the role of lipid dysregulation in other polyQ disorders remains less well defined. In this review, we summarize key lipid functions in the central nervous system, synthesize reported lipid alterations across polyQ diseases, and evaluate the therapeutic potential of targeting lipid metabolism.
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BibTeX
@article{ni2026lipid,
title = {{Lipid dysregulation in polyglutamine diseases}},
author = {Luhan Ni and Vaishnavi Veeranki and Byron Huang and Janghoo Lim},
journal = {Molecules and Cells},
year = {2026},
month = oct,
doi = {10.1016/j.mocell.2026.100406},
url = {https://doi.org/10.1016/j.mocell.2026.100406},
note = {CC BY-NC-ND 4.0},
}
RIS
TY - JOUR
TI - Lipid dysregulation in polyglutamine diseases
AU - Luhan Ni
AU - Vaishnavi Veeranki
AU - Byron Huang
AU - Janghoo Lim
PY - 2026
DA - 2026/10/14
JO - Molecules and Cells
DO - 10.1016/j.mocell.2026.100406
UR - https://doi.org/10.1016/j.mocell.2026.100406
AB - Polyglutamine (polyQ) diseases are fatal neurodegenerative disorders caused by pathological cytosine-adenine-guanine (CAG) repeat expansions in disease-associated genes. They are characterized by progressive motor dysfunction and typically late onset. Although multiple mechanisms have been proposed, accumulating evidence indicates that lipid dysregulation contributes to neuronal dysfunction and degeneration. Lipids are essential for brain development and homeostasis, and disrupted lipid metabolism is implicated across neurodegenerative conditions. Within the polyQ spectrum, Huntington’s disease is the best studied and shows strong links between altered lipid pathways and neuronal pathology, whereas the role of lipid dysregulation in other polyQ disorders remains less well defined. In this review, we summarize key lipid functions in the central nervous system, synthesize reported lipid alterations across polyQ diseases, and evaluate the therapeutic potential of targeting lipid metabolism.
SN - 1016-8478
SN - 0219-1032
ER -