Paola Podini

Berberine improves motor deficits in the spastic paraplegia SPG7 mutant mice

This study shows that berberine improves motor deficits in SPG7 mutant mice by modulating mitochondrial permeability transition pore dynamics.

Early endothelial activation at the blood-nerve barrier defines a hallmark of ALS

The paper identifies early endothelial activation at the blood-nerve barrier as a key feature of ALS, occurring before CNS vascular changes and motor neuron damage.

Glia-enriched stem-cell 3D model of the human brain mimics the glial-immune neurodegenerative phenotypes of multiple sclerosis

This study develops a glia-enriched 3D brain model from human iPSCs that mimics MS-related neurodegenerative phenotypes and intercellular communication.

Circulating mitochondrial bioenergetics as fingerprint of the hepatic one: how to monitor genetic MASLD

The paper investigates how genetic variants in PNPLA3, MBOAT7, and TM6SF2 affect mitochondrial function in MASLD, showing that restoring these proteins improves mitochondrial bioenergetics and that circulating respirometry reflects hepatic mitochondrial status.

Prostaglandin D2 synthase controls Schwann cells metabolism

This study shows that L-PGDS in Schwann cells maintains myelin integrity by altering metabolism and producing ketone bodies and acetate.

NEK1 haploinsufficiency impairs ciliogenesis in human iPSC-derived motoneurons and brain organoids

This study shows that NEK1 haploinsufficiency in human iPSC-derived motoneurons and brain organoids impairs ciliogenesis, linking NEK1 mutations to ALS through reduced primary cilium formation.

Two neuronal models of TDP-43 proteinopathy display reduced axonal translation, increased oxidative stress, and defective exocytosis.

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.

ZFP423 regulates early patterning and multiciliogenesis in the hindbrain choroid plexus

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