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This research investigates how c-Abl-RIPK3 signaling affects mitochondrial function and autophagy in Gaucher disease.

April 11, 2026AntioxidantsOpen Access

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

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

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.

Lamaizon et al. (2026) studied this question.

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