M. Laura Feltri

The STRIPAK complex is required for radial sorting and laminin receptor expression in Schwann cells

This study identifies striatin-3 as a Rac1 interactor and shows its role in Schwann cell radial sorting and Hippo pathway regulation during peripheral nervous system development.

Human iPSC-derived myelinating organoids andgloboid cells to study Krabbe Disease

This study uses iPSC-derived myelinating organoids and microglia from Krabbe Disease patients to investigate early myelination defects and globoid cell formation mechanisms.

Loss of prohibitin 2 in Schwann cells dysregulates key transcription factors controlling developmental myelination

This study shows that loss of Phb2 in Schwann cells disrupts myelination and dysregulates key transcription factors essential for peripheral nerve development.

p38γMAPK delays myelination and remyelination and is abundant in multiple sclerosis lesions

This study shows that p38γMAPK inhibits oligodendrocyte differentiation and myelin repair, and is enriched in multiple sclerosis lesions.

MPZ-T124M mouse model replicates human axonopathy and suggest alteration in axo-glia communication

The paper develops a P0T124M mouse model of CMT2J neuropathy, showing axonal damage without significant myelin defects and altered axo-glia communication.

mTORC1 and JUN are activated after deletion of Prohibitin 1 in Schwann cells and may link mitochondrial dysfunction to demyelination

This study shows that mTORC1 and JUN are activated in Schwann cells lacking Prohibitin 1, contributing to demyelination through mitochondrial dysfunction.

Age-related neurodegeneration and cognitive impairments of NRMT1 knockout mice are preceded by misregulation of RB and expansion of the neural stem cell population

This study shows that NRMT1 knockout mice develop neurodegeneration and cognitive impairments due to early misregulation of RB and expansion of neural stem cell populations.

Brainstem development requires galactosylceramidase and is critical for the pathogenesis of Krabbe Disease

This study shows that GALC is critical for brainstem development and KD pathogenesis, with a specific vulnerability period between postnatal days 4-6.

A nonsense mutation in Myelin Protein Zero causes congenital hypomyelination neuropathy through altered P0 membrane targeting and gain of abnormal function

Neuregulin 1 type III reduces severity in a mouse model of Congenital Hypomyelinating Neuropathy

This study shows that increasing neuregulin 1 type III in neurons improves myelination in a mouse model of congenital hypomyelinating neuropathy without relying on Egr2 or typical myelin genes.

Enhanced axonal Neuregulin-1 type-III signaling ameliorates disease severity in a CMT1B mouse model

This study shows that enhancing Nrg1TIII signaling reduces disease severity in a CMT1B mouse model by rebalancing myelin lipids and proteins, not through myelin gene upregulation.