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April 11, 2026AntioxidantsOpen Access

The c-Abl-RIPK3 Axis Drives Mitochondrial Dysfunction and Impaired Mitophagy in Gaucher Disease Models

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Authors

CLCristian M. LamaizonRTRenatta Tironi-HernándezNANohela B. Arévalo

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Overview

Demonstrates mitochondrial dysfunction in Gaucher disease, indicating new therapeutic targets for neuronopathic forms.

Key Points

  • This research investigates how c-Abl-RIPK3 signaling affects mitochondrial function and autophagy in Gaucher disease.
  • Used fibroblasts from GBA1 mutation patients and neurons treated with glucocerebrosidase inhibitor CBE.
  • Analyzed mitochondrial morphology and ΔΨm (mitochondrial membrane potential).
  • Assessed the capacity to form autophagosomes.
  • Performed pharmacological inhibition of c-Abl and RIPK3 to evaluate mitochondrial and autophagy restoration.
  • Mitochondria showed dysfunction and altered morphology in Gaucher disease models.
  • Pharmacological inhibition of c-Abl and RIPK3 restored mitochondrial function.
  • Inhibition increased autophagosome formation and enhanced mitochondrial engulfment in both models.

Cite This Study

Lamaizon et al. (2026) studied this question.

synapsesocial.com/papers/69d9e64e78050d08c1b76973https://doi.org/10.3390/antiox15040465
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