María José Castellanos‐Montiel

BetaII-Spectrin Gaps and Patches Emerge from the Patterned Assembly of the Actin/Spectrin Membrane Skeleton in Human Motor Neuron Axons

The paper uses imaging to study the spatial distribution of betaII-spectrin in human motor neuron axons, revealing a gap-and-patch pattern in the actin/spectrin membrane skeleton.

Applications of adeno-associated virus for 3D single-cell morphometric analysis in iPSC-derived midbrain organoids.

This study uses adeno-associated virus to image and analyze 3D morphology and connectivity of neurons and astrocytes in living human midbrain organoids over time.

Transcriptome-based screening in TARDBP/TDP-43 knock-in motor neurons identifies the NEDD8-activating enzyme inhibitor MLN4924

The paper identifies MLN4924 as a compound that improves cell viability in TDP-43 mutant motor neurons by targeting NEDD8-activating enzyme.

Mutations in SOD1 induce ALS-related phenotypes in 3D iPSC-derived motor neuron (MN) spheroids

This study uses 3D iPSC-derived motor neuron spheroids with SOD1 mutations to show ALS-related phenotypes and genetic variability effects.

Homozygous ALS-linked mutations in TARDBP/TDP-43 lead to hypoactivity and synaptic abnormalities in human iPSC-derived motor neurons

This study shows that homozygous ALS-linked TARDBP mutations cause synaptic abnormalities and reduced activity in human iPSC-derived motor neurons without affecting TDP-43 localization or aggregation.

KCC2 as a novel biomarker and therapeutic target for motoneuron degenerative disease

This study identifies KCC2 as an early biomarker and therapeutic target for ALS by showing its downregulation in motor neurons and the ability of KCC2-enhancing compounds to delay disease progression.

An optimized workflow to generate and characterize iPSC-derived motor neuron (MN) spheroids

This study develops a workflow to generate and analyze iPSC-derived motor neuron spheroids, confirming their marker expression and functional activity.

A streamlined CRISPR workflow to introduce mutations and generate isogenic iPSCs for modeling amyotrophic lateral sclerosis

This study introduces a streamlined CRISPR workflow to generate isogenic iPSCs with specific ALS-related mutations for modeling the disease.