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Developmental expression of the SRF co‐activator MAL in brain: role in regulating dendritic morphology
Findings indicate that the MAL‐SRF signaling pathway plays a key role in regulating dendritic morphology in adult brain and cultured cortical neurons.
Modeling sporadic ALS in iPSC-derived motor neurons identifies a potential therapeutic agent
iPSC-derived motor neurons from over 30 heterogeneous sporadic ALS cases exhibit pathologies correlated with clinical disease progression, are more similar to FUS/TDP-43 familial ALS than SOD1-ALS and are corrected by repurposing of ropinirole.
Aberrant axon branching via Fos-B dysregulation in FUS-ALS motor neurons
Analyzing the axonal fraction of hiPSC-derived MNs using microfluidic devices revealed that Fos-B is a key regulator of FUS-mutant axon branching, which is a novel phenotype and confirmed with other ALS causative mutation than FUS.
Involvement of the Serum Response Factor Coactivator Megakaryoblastic Leukemia (MKL) in the Activin-regulated Dendritic Complexity of Rat Cortical Neurons*
It is demonstrated that in addition to MKL1, MKL2, highly enriched in the forebrain, strongly contributes to the dendritic complexity, and this process is triggered by stimulation with activin, a member of the transforming growth factor β (TGF-β) superfamily.
Neuron-enriched phosphatase and actin regulator 3 (Phactr3)/ nuclear scaffold-associated PP1-inhibiting protein (Scapinin) regulates dendritic morphology via its protein phosphatase 1-binding domain.
Results showed that Phactr3/Scapinin expression was up-regulated in the developing brain and enriched in neurons and in the postsynaptic density fraction, but not in astrocytes, and suggested it might contribute to synaptic formation via distinct actin- and PP1-binding domains involved in dendritic and axonal morphology, respectively.
Establishment of In Vitro FUS-Associated Familial Amyotrophic Lateral Sclerosis Model Using Human Induced Pluripotent Stem Cells
Summary Amyotrophic lateral sclerosis (ALS) is a late-onset motor neuron disorder. Although its neuropathology is well understood, the cellular and molecular mechanisms are yet to be elucidated due
Differential gene expression profiles in neurons generated from lymphoblastoid B-cell line-derived iPS cells from monozygotic twin cases with treatment-resistant schizophrenia and discordant
A new strategy is established for the use of monozygotic twin studies in schizophrenia research by generating neurons from iPS cells derived from patients with treatment-resistant schizophrenia and performing RNA-sequencing to compare the transcriptome profiles of the mock or clozapine-treated neurons.
Functional Neurons Generated from T Cell-Derived Induced Pluripotent Stem Cells for Neurological Disease Modeling
A neurosphere-based robust differentiation protocol was developed, which enabledTiPSCs to differentiate into functional neurons, despite differences in global gene expression between TiPSCs and adult human dermal fibroblast-derived iPSCs.
The pathogenesis linked to coenzyme Q10 insufficiency in iPSC-derived neurons from patients with multiple-system atrophy
Functional deficiencies in mitochondrial respiration and the antioxidative system in induced pluripotent stem cell-derived neurons from an MSA patient with compound heterozygous COQ2 mutations are reported and may contribute to the development of therapy using coenzyme Q10 supplementation.
Brain Transcriptome Analysis Links Deficiencies of Stress-Responsive Proteins to the Pathomechanism of Kii ALS/PDC
To gather new insights into the pathological mechanisms underlying Kii ALS/PDC, transcriptome analyses of patient brains revealed that expression levels of genes associated with heat shock proteins, DNA binding/damage, and senescence were significantly altered in patients with ALS/PB compared with healthy individuals.