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Investigation of space charge accumulation processes in a chemically cross-linked polyethylene for ac usage (XLPE) and a modified XLPE for dc usage (DC-XLPE) at voltage polarity reversal was carried out using pulsed electro-acoustic (PEA) measurement system. We found that a huge amount of so called packet-like charge, some of which partially increased the electric field in the material to more than twice of the average applied stress, generated in XLPE at the polarity reversal of DC stress. It enhanced the electric stress locally in the sample by almost twice of the applied average stress. On the other hand, we also found that the packet-like charge was not observed in DC-XLPE under the same experimental condition. [ABSTRACT FROM PUBLISHER]

Targeted genome editing has made significant advancements; however, safety and ethical issues have not been fully elucidated, resulting in strict control of the technique. We tested genome editing tools on gametes from a genetically humanized mouse model using a phenylketonuria (PKU) mouse model to gain insights into genome editing in human embryos. The human PKU mouse model Pah hR111X mice was generated. The junctional region between exon 3 and intron 3 of Pah was replaced with a 120 bp corresponding human PAH sequence, including the pathogenic common variant c.331C > T in Pah hR111X mice. Pah hR111X mice successfully recapitulated the PKU phenotype and showed cognitive dysfunction and depressive-like behavior, which are observed in human patients with PKU. Genome editing was applied to fertilized eggs of Pah hR111X mice utilizing sgRNA that targets the human sequence. Mice with the corrected allele exhibited normal serum phenylalanine levels. Through genome editing, we validated the utility of sgRNA. The genetically humanized mouse model suggested that germ-line genome editing of the pathogenic variant may be feasible for monogenic disorders by revealing the recovery of the phenotype; however, there are remaining issues with the tool, including its efficiency and accuracy. This genome editing protocol using a genetically humanized mouse model will provide insights for improving current issues and contribute to the establishment of heritable human genome editing protocols., (© 2024 SSIEM.)