Transmit and protect: The mechanical functions of intermediate filaments

Pogoda, Katarzyna; Janmey, Paul A

doi:10.1016/j.ceb.2023.102281

Cited by 3 publications

(1 citation statement)

References 33 publications

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“…IFs play an important role in maintaining the mechanical and viscoelastic properties of cells (Sivaramakrishnan et al, 2008), which are important for a wide range of cellular functions, such as cell migration, division, and differentiation. IFs provide structural support and help to maintain cell and tissue shape, especially under mechanical stress or strain (Pogoda & Janmey, 2023). Additionally, intermediate filaments also act as scaffolds involved in cell signalling and in the localization and traffic of organelles, such as mitochondria, lysosomes and other vesicles.…”

Section: Introductionmentioning

confidence: 99%

Subcellular, biochemical and biophysical alterations in two glial cell models of ARSACS

Murtinheira,

Boasinha,

Belo

et al. 2024

Preprint

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Autosomal recessive spastic ataxia of Charlevoix-Saguenay (ARSACS) is a developmental and degenerative disorder caused by loss-of-function mutations in the gene that codifies for the sacsin chaperone. Sacsin was initially described as a neuronal protein but is found in various cell types, including astroglial, microglial, kidney, and skin cell lines. We and others have shown that virtually all cell and animal models of ARSACS show disruption of intermediate filament (IF) cytoskeleton and organelle distribution. This article extends previous observations from our lab on the C6 astroglial-like model and describes the development and characterization of a new human microglial cell model based on HMC3 cells. HMC3 cells knocked out for sacsin show similar alterations to C6 astroglial-like cells: aberrant distribution of IF network and organelles, downregulation of developmental transcription factors STAT3 and Smad1, and alterations in autophagy machinery and markers of intracellular stress, resulting in morphological changes. Our results extend previous observations suggesting a possible role for glial cells in ARSACS and provide new tools to understand the glial-specific mechanisms involved in this pathology.

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Section: Introductionmentioning

confidence: 99%

Subcellular, biochemical and biophysical alterations in two glial cell models of ARSACS

Murtinheira,

Boasinha,

Belo

et al. 2024

Preprint

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A human microglial cell model of autosomal recessive spastic ataxia of Charlevoix-Saguenay

Murtinheira,

Farsetti,

Macedo

et al. 2024

Biochimica et Biophysica Acta (BBA) - Molecular Basis of Diseas

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circRNAs as Epigenetic Regulators of Integrity in Blood–Brain Barrier Architecture: Mechanisms and Therapeutic Strategies in Multiple Sclerosis

D’Aversa,

Salvatori,

Vaccarezza

et al. 2024

Cells

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Multiple sclerosis (MS) is a chronic inflammatory neurodegenerative disease leading to progressive demyelination and neuronal loss, with extensive neurological symptoms. As one of the most widespread neurodegenerative disorders, with an age onset of about 30 years, it turns out to be a socio-health and economic issue, thus necessitating therapeutic interventions currently unavailable. Loss of integrity in the blood–brain barrier (BBB) is one of the distinct MS hallmarks. Brain homeostasis is ensured by an endothelial cell-based monolayer at the interface between the central nervous system (CNS) and systemic bloodstream, acting as a selective barrier. MS results in enhanced barrier permeability, mainly due to the breakdown of tight (TJs) and adherens junctions (AJs) between endothelial cells. Specifically, proinflammatory mediator release causes failure in cytoplasmic exposure of junctions, resulting in compromised BBB integrity that enables blood cells to cross the barrier, establishing iron deposition and neuronal impairment. Cells with a compromised cytoskeletal protein network, fiber reorganization, and discontinuous junction structure can occur, resulting in BBB dysfunction. Recent investigations on spatial transcriptomics have proven circularRNAs (circRNAs) to be powerful multi-functional molecules able to epigenetically regulate transcription and structurally support proteins. In the present review, we provide an overview of the recent role ascribed to circRNAs in maintaining BBB integrity/permeability via cytoskeletal stability. Increased knowledge of the mechanisms responsible for impairment and circRNA’s role in driving BBB damage and dysfunction might be helpful for the recognition of novel therapeutic targets to overcome BBB damage and unrestrained neurodegeneration.

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Transmit and protect: The mechanical functions of intermediate filaments

Cited by 3 publications

References 33 publications

Subcellular, biochemical and biophysical alterations in two glial cell models of ARSACS

Subcellular, biochemical and biophysical alterations in two glial cell models of ARSACS

A human microglial cell model of autosomal recessive spastic ataxia of Charlevoix-Saguenay

circRNAs as Epigenetic Regulators of Integrity in Blood–Brain Barrier Architecture: Mechanisms and Therapeutic Strategies in Multiple Sclerosis

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