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Related Concept Videos

Parkinson Disease ll: Pathophysiology01:24

Parkinson Disease ll: Pathophysiology

Parkinson disease (PD) is a progressive neurodegenerative disorder primarily affecting movement, with additional non-motor features. Its pathophysiology involves complex interactions among genetic susceptibility, environmental exposures, and cellular dysfunction, including dopaminergic neuron loss, protein aggregation, and mitochondrial impairment.Selective NeurodegenerationA key feature is the degeneration of dopaminergic neurons in the substantia nigra pars compacta, leading to reduced...
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Related Experiment Video

Updated: May 14, 2026

In Vitro Aggregation Assays Using Hyperphosphorylated Tau Protein
09:22

In Vitro Aggregation Assays Using Hyperphosphorylated Tau Protein

Published on: January 2, 2015

Cell Cycle Dysfunction and Metabolic Alterations Consistent With Pathological Tau in Progressive Supranuclear Palsy

Roberta De Mori1, Francesca Ciaiola2,3, Valentina Nesci4,5

  • 1Induced Pluripotent Stem Cells and Organoids Unit IRCCS Santa Lucia Foundation, Via Ardeatina 306-354, Rome, 00179, Italy.

Molecular Neurobiology
|May 12, 2026
PubMed
Summary

Progressive supranuclear palsy (PSP) patient fibroblasts show altered cell cycle dynamics and increased mitochondrial function. These findings highlight fibroblasts as a valuable model for studying PSP and potential therapeutic targets.

Keywords:
Cell cycleFibroblastsProgressive supranuclear palsyProteomeStress granule

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Last Updated: May 14, 2026

In Vitro Aggregation Assays Using Hyperphosphorylated Tau Protein
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Using Live-Cell Imaging to Measure the Effects of Pathological Proteins on Axonal Transport in Primary Hippocampal Neurons
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Using Live-Cell Imaging to Measure the Effects of Pathological Proteins on Axonal Transport in Primary Hippocampal Neurons

Published on: December 22, 2023

Area of Science:

  • Neuroscience
  • Cell Biology
  • Genetics

Background:

  • Progressive supranuclear palsy (PSP) is a neurodegenerative disorder characterized by Tau protein aggregation.
  • Current animal models do not fully replicate PSP's cytopathological features.
  • Investigating patient-derived cells offers a promising avenue for understanding PSP.

Purpose of the Study:

  • To identify biological patterns in primary dermal fibroblasts from PSP patients.
  • To explore cell cycle dynamics, mitochondrial homeostasis, and proteomic profiles in PSP fibroblasts.
  • To evaluate fibroblasts as a model for PSP research and drug development.

Main Methods:

  • Collected primary dermal fibroblasts from 7 PSP patients and 4 healthy donors.
  • Analyzed cell proliferation rates and expression of cell cycle checkpoints (p16/p21).
  • Investigated stress granule formation (G3BP1) associated with Tau, mitochondrial homeostasis, and proteomic profiles.

Main Results:

  • PSP fibroblasts exhibited slower proliferation and increased p16/p21 expression, indicating cell cycle alterations.
  • Increased association of Tau with G3BP1-positive stress granules was observed.
  • Altered mitochondrial homeostasis and differential proteomic clustering were noted in PSP cells.
  • Evidence suggests increased mitochondrial function in PSP fibroblasts, potentially a cellular coping mechanism.

Conclusions:

  • Cell cycle dynamics and senescence are significantly altered in PSP.
  • Primary fibroblasts serve as a reliable patient-derived model for studying PSP pathology.
  • Fibroblasts offer a platform for testing therapeutic compounds for PSP.
  • Mitochondrial function may represent an adaptive response in diseased cells.