G. Giacomo Consalez

This study investigates how TDP-43 dysregulation disrupts protein synthesis in ALS models by altering ribosome dynamics and mRNA homeostasis, revealing a conserved mechanism across in vitro and in vivo systems.

The paper identifies a novel subset of cerebellar nuclei neurons in mice that originate from an extrinsic germinal zone, distinct from the rhombic lip and ventricular zone.

This study investigates FXN depletion in cerebellar astrocytes, revealing altered calcium responses and antioxidant properties that may contribute to neurodegeneration in Friedreich's ataxia.

This study creates a PrknR275W mouse model of ARJP, showing early dopamine neuron dysfunction, age-dependent loss, and motor impairment, recapitulating key features of juvenile Parkinsonism.

This study characterizes two TDP-43 proteinopathy models in mouse neurons, revealing reduced axonal translation, increased oxidative stress, and presynaptic dysfunction linked to mRNA metabolism disruption.

This study maps the spatial-temporal development of glutamatergic neurons in embryonic cerebellar nuclei, revealing significant cellular heterogeneity and distinct subpopulations.

This study shows that ZFP423 is essential for hindbrain choroid plexus development, regulating early patterning and multiciliogenesis through gene expression and cell fate specification.

This study identifies the role of COE-type transcription factors in axial motor neuron development across species, showing functional conservation and specific requirements for Ebf1 and Ebf2 in mice and nematodes.

This study investigates the role of Zfp423 in cerebellar neurogenesis, revealing its domain-specific effects on cell cycle progression, DNA damage response, and Purkinje cell development in mouse models.