Connexin 30 (Cx30), which forms gap junctions between astrocytes, regulates cell adhesion and migration, and modulates glutamate transport. Cx30 is upregulated on activated astroglia in central nervous system inflammatory lesions, including spinal cord lesions in mutant superoxide dismutase 1 (mSOD1) transgenic amyotrophic lateral sclerosis (ALS) model mice. Here, we investigated the role of Cx30 in mSOD1 mice. Cx30 was highly expressed in the pre-onset stage in mSOD1 mice. mSOD1 mice with knockout (KO) of the Cx30 gene (Cx30KO-mSOD1 mice) showed delayed disease onset and tended to have an extended survival period (log-rank, p = 0.09). At the progressive and end stages of the disease, anterior horn cells were significantly preserved in Cx30KO-mSOD1 mice. In lesions of these mice, glial fibrillary acidic protein/C3-positive inflammatory astroglia were decreased. Additionally, the activation of astrocytes in Cx30KO-mSOD1 mice was reduced compared with mSOD1 mice by gene expression microarray. Furthermore, expression of connexin 43 at the pre-onset stage was downregulated in Cx30KO-mSOD1 mice. These findings suggest that reduced expression of astroglial Cx30 at the early disease stage in ALS model mice protects neurons by attenuating astroglial inflammation.

Background: Decentralized clinical trials are becoming more widespread, driven by advances in digital technologies. However, little is known about the use of these trials in Japan. Methods: We carried out a nationwide survey targeting 221 major clinical research institutions in Japan. Respondents were asked to report details of investigator-initiated decentralized clinical trials for which their institution was the lead facility for fiscal years 2021–2025. Results: Overall, 82 institutions responded (37.1% response rate), including all 54 Clinical Research Core Hospitals and National University Hospitals (100% response rate), which are the institutions most likely to conduct investigator-initiated clinical research. Eighteen institutions (22.0%) reported conducting a total of 36 decentralized clinical trials, mostly at Clinical Research Core Hospitals or National University Hospitals. Twenty-one trials (58.3%) were classified as interventional studies under the Ethical Guidelines. Annual initiations increased from four in fiscal year 2021 to 15 in fiscal year 2024, and seven were reported for the incomplete fiscal year 2025. The most frequently used decentralized elements were electronic informed consent (eConsent) (14 trials, 38.9%) and online medical consultations (12 trials, 33.3%). Twenty-seven trials (75.0%) incorporated only one or two decentralized elements. Oncology was the most common disease area, although the trials spanned a wide range of therapeutic areas. Conclusions: The use of decentralized clinical trials is gradually increasing in Japan. These trials often use hybrid approaches incorporating a few decentralized elements, such as eConsent and online medical consultations. This pattern may reflect an approach adapted to Japan’s unique regulatory, healthcare, and geographical circumstances.

Objective: Needle electromyography (nEMG) is essential for diagnosing neuromuscular disorders but is invasive and often painful. We employed single-channel bipolar surface EMG (sEMG) analyzed with a novel wavelet-based analytical approach, “Detecting and Extracting Elemental Wave Components based on a Wavelet Coefficient Set (DEWCS)” and investigated whether fasciculation-related activity could be identified. Methods: In this prospective study, 28 patients undergoing nEMG for suspected neuromuscular disorders and 13 healthy controls were included. Resting-state sEMG was recorded from selected muscles using single-channel bipolar active electrodes at a high sampling rate. DEWCS was used to extract indices reflecting fast- and slow-type motor unit (MU)-related activity. These standardized indices were evaluated against nEMG-detected fasciculation potentials using generalized estimating equation logistic regression to account for within-subject clustering. Diagnostic performance was assessed by receiver operating characteristic analysis. Results: A total of 67 muscles from 38 participants were analyzed. Indices of fast- and slow-type MU-related activity were significantly associated with fasciculation potentials (slow: OR 5.10, p = 0.0041; fast: OR 2.38, p = 0.0162). The combined model showed excellent discrimination (area under the curve = 0.97), outperforming either index alone. Muscle region had no significant effect. Conclusions: A single-channel bipolar sEMG setup combined with DEWCS detected fasciculation-related activity with promising accuracy. This method may serve as a non-invasive surrogate marker of lower motor neuron involvement. Further validation in larger cohorts is warranted. Significance: This non-invasive sEMG approach may help detect fasciculation-related activity and complement nEMG in neuromuscular diagnostics.