Progressive Degeneration of Human Neural Stem Cells Caused by Pathogenic LRRK2 Researchers reported on the generation of induced pluripotent stem cells derived from Parkinson’s disease patients and the implications of leucine-rich repeat kinase 2 (LRRK2)(glycine-to-serine substitution at amino acid 2019) mutation in human neural-stem-cell populations. [Nature] Abstract | Press Release DICER- and AGO3-Dependent Generation of Retinoic Acid-Induced DR2 Alu RNAs Regulates Human Stem Cell Proliferation Investigators report that ~2-3% of the ~100,000-200,000 total human DR2 Alu repeats located in proximity to activated polymerase II (Pol II) transcription units are activated by the retinoic acid receptor in human embryonic stem cells to generate Pol III-dependent RNAs. [Nat Struct Mol Biol] Abstract Induced Pluripotent Stem Cell Model Recapitulates Pathologic Hallmarks of Gaucher Disease To model Gaucher disease (GD), researchers generated human induced pluripotent stem cells, by reprogramming skin fibroblasts from patients with type 1, type 2, and type 3 GD. [Proc Natl Acad Sci USA] Abstract | Full Article | Press Release Exogenous Activation of BMP-2 Signaling Overcomes TGFβ-Mediated Inhibition of Osteogenesis in Marfan Embryonic Stem Cells and Marfan Patient Specific Induced-Pluripotent Stem Cells Researchers have previously demonstrated that unique skeletal phenotypes observed in human embryonic stem cells carrying the monogenic Fibrillin-1 (FBN1) mutation (MFS cells) are faithfully phenocopied by cells differentiated from induced pluripotent-stem cells (MFSiPS) derived independently from MFS patient fibroblasts. In the current study, the authors aimed to determine further the biochemical features of transducing signaling(s) in MFS stem cells and MFSiPS cells highlighting a cross-talk between TGFβ and BMP signaling. [Stem Cells] Abstract The Positional Identity of Mouse ES Cell-Generated Neurons Is Affected by BMP Signaling Scientists investigated the effects of bone morphogenetic proteins (BMPs) in determining the positional identity of neurons generated in vitro from mouse embryonic stem (ES) cells. [Cell Mol Life Sci] Abstract | Full Article Transplantation of Parthenogenetic Embryonic Stem Cells Ameliorates Cardiac Dysfunction and Remodeling after Myocardial Infarction Researchers investigated whether parthenogenetic embryonic stem cells (pESCs) could repair myocardial infarction as compared to ESCs. [Cardiovasc Res] Abstract Medaka Embryonic Stem Cells Are Capable of Generating Entire Organs and Embryo-Like Miniatures Researchers presented data in the fish medaka that embryonic stem cells have a novel ability to form extra organs and even embryo-like miniatures. [Stem Cells Dev] Abstract Targeted Genomic Integration of a Selectable Floxed Dual Fluorescence Reporter in Human Embryonic Stem Cells Investigators used zinc finger nuclease technology to stably insert a unique, selectable, floxed dual-fluorescence reporter transgene into the AAVS1 locus of RUES2 human embryonic stem cells. [PLoS One] Full Article Bone Tissue Engineering of Induced Pluripotent Stem Cells Cultured with Macrochanneled Polymer Scaffold Human-induced pluripotent stem cells without an embryoid body step were cultured in macrochanneled poly(caprolactone) scaffolds prepared using a robotic dispensing technique, after which osteogenesis was promoted by the addition of exogenous osteogenic factors. [J Biomed Mater Res A] Abstract Induced Pluripotent Stem Cell Models of Progranulin-Deficient Frontotemporal Dementia Uncover Specific Reversible Neuronal Defects Scientists generated multiple induced pluripotent stem cell lines from a control subject, a patient with sporadic frontotemporal dementia (FTD), and an FTD patient with a novel heterozygous GRN mutation (progranulin [PGRN] S116X). Findings identify cell-autonomous, reversible defects in patient neurons with PGRN deficiency, and provide a compelling model for studying PGRN-dependent pathogenic mechanisms and testing potential therapies. [Cell Rep] Abstract |