Role of glial cells in motor neuron degeneration in hereditary spastic paraplegias DOI Creative Commons

Manaswini Vijayaraghavan,

S. R. Murali, Gitika Thakur

et al.

Frontiers in Cellular Neuroscience, Journal Year: 2025, Volume and Issue: 19

Published: April 15, 2025

This review provides a comprehensive overview of hereditary spastic paraplegias (HSPs) and summarizes the recent progress on role glial cells in pathogenesis HSPs. HSPs are heterogeneous group neurogenetic diseases characterized by axonal degeneration cortical motor neurons, leading to muscle weakness atrophy. Though contribution cells, especially astrocytes, progression other neuron like amyotrophic lateral sclerosis (ALS) is well documented, interaction between neurons astrocytes HSP remained unknown until recently. Using human pluripotent stem cell-based models HSPs, study reported impaired lipid metabolisms reduced size droplets astrocytes. Moreover, targeting dysfunction rescues demonstrating non-cell-autonomous mechanism deficits neurons. In addition studies revealed dysfunctions patient cell-derived microglial cells. Increased microgliosis pro-inflammation factors were also observed patients’ samples, pointing an exciting innate immunity microglia HSP. Building upon these studies, further investigation detailed molecular interplay cell neuronal combining models, animal samples will open avenues for identifying new therapeutic targets strategies

Language: Английский

Role of glial cells in motor neuron degeneration in hereditary spastic paraplegias DOI Creative Commons

Manaswini Vijayaraghavan,

S. R. Murali, Gitika Thakur

et al.

Frontiers in Cellular Neuroscience, Journal Year: 2025, Volume and Issue: 19

Published: April 15, 2025

This review provides a comprehensive overview of hereditary spastic paraplegias (HSPs) and summarizes the recent progress on role glial cells in pathogenesis HSPs. HSPs are heterogeneous group neurogenetic diseases characterized by axonal degeneration cortical motor neurons, leading to muscle weakness atrophy. Though contribution cells, especially astrocytes, progression other neuron like amyotrophic lateral sclerosis (ALS) is well documented, interaction between neurons astrocytes HSP remained unknown until recently. Using human pluripotent stem cell-based models HSPs, study reported impaired lipid metabolisms reduced size droplets astrocytes. Moreover, targeting dysfunction rescues demonstrating non-cell-autonomous mechanism deficits neurons. In addition studies revealed dysfunctions patient cell-derived microglial cells. Increased microgliosis pro-inflammation factors were also observed patients’ samples, pointing an exciting innate immunity microglia HSP. Building upon these studies, further investigation detailed molecular interplay cell neuronal combining models, animal samples will open avenues for identifying new therapeutic targets strategies

Language: Английский

Citations

0