Accumulation of ether phospholipids in induced pluripotent stem cells and oligodendrocyte-lineage cells established from patients with Sjögren-Larsson syndrome.

Sjögren-Larsson syndrome (SLS) is an autosomal recessive leukodystrophy characterized by ichthyosis, intellectual disability, and progressive spastic paralysis caused by biallelic pathogenic variants in the ALDH3A2 gene that encodes the fatty aldehyde dehydrogenase, fatty aldehyde dehydrogenase (FALDH); FALDH catalyzes several metabolic reactions involved in fatty aldehyde oxidation. Only a few studies have been performed to determine the lipid profile of patients with SLS. In a previous postmortem study of the brain of a 65-year-old patient with SLS, lipidomic analysis revealed an accumulation of long-chain unsaturated ether lipid species in the white matter and gray matter.

Real-world outcomes of avelumab plus axitinib in patients with advanced renal cell carcinoma in Japan: long-term follow-up from the J-DART2 retrospective study.

Background: Avelumab + axitinib was approved for advanced renal cell carcinoma (aRCC) in Japan in December 2019. We report long-term real-world outcomes with first-line avelumab + axitinib from the J-DART2 study in Japan.

Methods: J-DART2 was a multicenter, noninterventional, retrospective study examining clinical data from patients with curatively unresectable locally advanced or metastatic RCC who started treatment with first-line avelumab + axitinib in Japan between December 2019 and October 2022.

Ultra-low noise measurements of ionic transport within individual single-walled carbon nanotubes.

Despite 15 years of extensive investigation, the fabrication and study of nanofluidic devices that incorporate a single carbon nanotube (CNT) still represents a remarkable experimental challenge. In this study, we present the fabrication of nanofluidic devices that integrate an individual single-walled CNT (SWCNT), showcasing a notable reduction in noise by 1-3 orders of magnitude compared to conventional devices. This achievement was made possible by employing high dielectric constant materials for both the substrate and the CNT-covering layer.

Epstein-Barr virus-positive plasmacytoma in an immunocompetent female: A case report.

Plasmacytoma is defined as a plasma cell neoplasm forming a solitary osseous or extramedullary tumor without evidence of myeloma or organ damage related to a plasma cell neoplasm. Epstein-Barr virus (EBV) is associated with various B-cell neoplasms, particularly in patients with immune dysregulation; however, plasmacytoma is typically negative for EBV. Here, a case of EBV-positive sternal plasmacytoma in an immunocompetent female is presented.

Atomic-Level Structure of the Organic-Inorganic Interface of Colloidal ZnO Nanoplatelets from Dynamic Nuclear Polarization-Enhanced NMR.

Colloidal semiconductor nanoplatelets (NPLs) have emerged as a new class of nanomaterials that can exhibit substantially distinct optical properties compared to those of isotropic quantum dots, which makes them prime candidates for new-generation optoelectronic devices. Insights into the structure and anisotropic growth of NPLs can offer a blueprint for their controlled fabrication. Here, we present an atomic-level investigation of the organic-inorganic interface structure in ultrathin and stable benzamidine ()-supported ZnO NPLs prepared by the modified ne-pot elf-upporting rganoetallic approach.