IntroductionBone abnormalities, one of the primary manifestations of Menkes disease (MD), include a weakened bone matrix and low mineral density. However, the molecular and cellular mechanisms underlying these bone defects are poorly understood.MethodsWe present in vitro modeling for impaired osteogenesis in MD using human induced pluripotent stem cells (iPSCs) with a mutated ATP7A gene. MD-iPSC lines were generated from two patients harboring different mutations.ResultsThe MD-iPSCs showed a remarkable retardation in CD105 expression with morphological anomalies during development to mesenchymal stem cells (MSCs) compared with wild-type (WT)-iPSCs. Interestingly, although prolonged culture enhanced CD105 expression, mature MD-MSCs presented with low alkaline phosphatase activity, reduced calcium deposition in the extracellular matrix, and downregulated osteoblast-specific genes during osteoblast differentiation in vitro. Knockdown of ATP7A also impaired osteogenesis in WT-MSCs. Lysyl oxidase activity was also decreased in MD-MSCs during osteoblast differentiation.ConclusionsOur findings indicate that ATP7A dysfunction contributes to retardation in MSC development and impairs osteogenesis in MD.Electronic supplementary materialThe online version of this article (doi:10.1186/s13287-015-0147-5) contains supplementary material, which is available to authorized users.

This study presents a new TEMPEST threat that an attacker can surreptitiously obtain original plain audio information from a distance by exploiting recently emerging unintentional electromagnetic (EM) radiations. As lightweight sensor-based Internet of things (IoT) services become widespread, a mixed-signal system on chip (MSoC) spontaneously integrates all components, such as digital, analog, and even power circuits, into a single chipset to minimize

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Using improper wound care materials may cause impaired wound healing, which can involve scar formation and infection. Herein, we propose a facile method to fabricate a cellalignment scaffold, which can effectively enhance cell growth and migration, leading to the reproduction of cellular arrangements and restoration of tissues. The principle is scratching a diamond lapping film that gives uniaxial nanotopography on substrates. Cells are seeded to follow the geometric cue via contact guidance, resulting in highly oriented cell alignment. Remarkable biocompatibility is also demonstrated by the high cell viability on various substrates. In vivo studies in a wound healing model in mice show that the scratched film supports directed cell guidance on the nanostructure, with significantly reduced wound areas and inhibition of excessive collagen deposition. Rapid recovery of the epidermis and dermis is also shown by histological analyses, suggesting the potential application of the scratching technique as an advanced wound dressing material for effective tissue regeneration.

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