Subject collection: Stem Cells
- mTOR hyperactivity mediates lysosomal dysfunction in Gaucher's disease iPSC-neuronal cells
Summary: Using neuronopathic Gaucher's disease iPSCs, this study has identified the mTOR complex as a potential therapeutic target in GBA1-associated neurodegeneration.
- C9ORF72-related cellular pathology in skeletal myocytes derived from ALS-patient induced pluripotent stem cells
Summary: Evidence of protein aggregation and mitochondrial dysfunction were found in skeletal myocytes differentiated from ALS-patient induced pluripotent stem cells with the C9ORF72 mutation.
- Genetic variation in GNB5 causes bradycardia by augmenting the cholinergic response via increased acetylcholine-activated potassium current (IK,ACh)
Summary: The authors show hiPSC-cardiomyocyte-based modeling of a mutation in a G-protein β-subunit that affects the electrophysiological response of cardiomyocytes to the cholinergic regulation of the heart rate, uncovering a potential therapy for severe bradycardia in humans carrying these mutations.
- Modeling blood diseases with human induced pluripotent stem cells
Summary: This Review discusses the applications of human iPSCs as preclinical models of cell and gene therapy for inherited monogenic blood disorders, as well as their more recent use as models of myeloid malignancies.
- Recapitulating Parkinson's disease pathology in a three-dimensional human neural cell culture model
Editor's choice: This study describes a novel 3D cell culture model that recapitulates key features of neuropathology in synucleinopathies, including large, nuclear-associated α-synuclein and ubiquitin-positive inclusions.
- 17-DMAG regulates p21 expression to induce chondrogenesis in vitro and in vivo
Summary: p21 inhibition in response to 17-DMAG treatment induces chondrogenesis in human synovial mesenchymal stem cells and promotes auricular cartilage regeneration in vivo.
- A nonalcoholic fatty liver disease model in human induced pluripotent stem cell-derived hepatocytes, created by endoplasmic reticulum stress-induced steatosis
Summary: Our study demonstrates expanded use of human induced pluripotent stem cell-derived hepatocytes for molecular studies and drug screening, to evaluate new therapeutics with an antisteatotic mechanism of action for nonalcoholic fatty liver disease.
- Plasticity of differentiated cells in wound repair and tumorigenesis, part II: skin and intestine
Summary: This final installment of a two-part Review discusses how cycles of dedifferentiation and redifferentiation can promote tumorigenesis in the skin and intestine, showing how plasticity in these continuously renewing tissues might contribute to tumorigenesis.
- Plasticity of differentiated cells in wound repair and tumorigenesis, part I: stomach and pancreas
Summary: Epithelial cells in diverse tissues have an innate plasticity program (recently dubbed ‘paligenosis’) such that differentiated cells can become regenerative upon large-scale insults to an organ. Thus, the proliferating, regenerative cells in an organ may not always derive directly from stem cells, which invites us to refine theories about the cell of origin of cancer such as the multi-hit model of tumorigenesis.
- Divergent effects of adrenaline in human induced pluripotent stem cell-derived cardiomyocytes obtained from hypertrophic cardiomyopathy
Summary: Different concentrations of adrenaline have divergent effects during and immediately after administration in human induced pluripotent stem cell-derived cardiomyocytes (hiPSC-CMs) obtained from hypertrophic cardiomyopathy (HCM) patients. Bisoprolol could not reduce the arrhythmias in HCM-specific hiPSC-CMs.