Antioxidants restore store-operated Ca entry in patient-iPSC-derived myotubes with tubular aggregate myopathy-associated Ile484ArgfsX21 STIM1 mutation via upregulation of binding immunoglobulin protein.

Store-operated Ca entry (SOCE) is indispensable for intracellular Ca homeostasis in skeletal muscle, and constitutive activation of SOCE causes tubular aggregate myopathy (TAM). To understand the pathogenesis of TAM, we induced pluripotent stem cells (iPSCs) from a TAM patient with a rare mutation (c.1450_1451insGA; p.

COVID-19 findings revealed via otolaryngological examination: Findings of a Japan Otorhinolaryngologist Association questionnaire.

Objective: In Japan, many otolaryngologists provide primary care for patients with coronavirus disease 2019 (COVID-19). The purpose of this study was to analyze the characteristics of otorhinolaryngological findings in order to improve COVID-19 diagnostic systems in a primary care setting.

Methods: A total of 351 patients (mean age, 36.

Pharmacological activation of SERCA ameliorates dystrophic phenotypes in dystrophin-deficient mdx mice.

Duchenne muscular dystrophy (DMD) is an X-linked genetic disorder characterized by progressive muscular weakness because of the loss of dystrophin. Extracellular Ca2+ flows into the cytoplasm through membrane tears in dystrophin-deficient myofibers, which leads to muscle contracture and necrosis. Sarco/endoplasmic reticulum Ca2+-ATPase (SERCA) takes up cytosolic Ca2+ into the sarcoplasmic reticulum, but its activity is decreased in dystrophic muscle.

iNOS is not responsible for RyR1 S-nitrosylation in mdx mice with truncated dystrophin.

Background: Previous research indicated that nitric oxide synthase (NOS) is the key molecule for S-nitrosylation of ryanodine receptor 1 (RyR1) in DMD model mice (mdx mice) and that both neuronal NOS (nNOS) and inducible NOS (iNOS) might contribute to the reaction because nNOS is mislocalized in the cytoplasm and iNOS expression is higher in mdx mice. We investigated the effect of iNOS on RyR1 S-nitrosylation in mdx mice and whether transgenic expression of truncated dystrophin reduced iNOS expression in mdx mice or not.

Methods: Three- to 4-month-old C57BL/6 J, mdx, and transgenic mdx mice expressing exon 45-55-deleted human dystrophin (Tg/mdx mice) were used.

Prostaglandin EP2 receptor downstream of Notch signaling inhibits differentiation of human skeletal muscle progenitors in differentiation conditions.

Understanding the signaling pathways that regulate proliferation and differentiation of muscle progenitors is essential for successful cell transplantation for treatment of Duchenne muscular dystrophy. Here, we report that a γ-secretase inhibitor, DAPT (N-[N-(3,5-difluorophenacetyl-L-alanyl)]-S-phenylglycine tertial butyl ester), which inhibits the release of NICD (Notch intercellular domain), promotes the fusion of human muscle progenitors in vitro and improves their engraftment in the tibialis anterior muscle of immune-deficient mice. Gene expression analysis revealed that DAPT severely down-regulates PTGER2, which encodes prostaglandin (PG) E2 receptor 2 (EP2), in human muscle progenitors in the differentiation condition.

Premyogenic progenitors derived from human pluripotent stem cells expand in floating culture and differentiate into transplantable myogenic progenitors.

Human induced pluripotent stem cells (hiPSCs) are a potential source for cell therapy of Duchenne muscular dystrophy. To reliably obtain skeletal muscle progenitors from hiPSCs, we treated hiPS cells with a Wnt activator, CHIR-99021 and a BMP receptor inhibitor, LDN-193189, and then induced skeletal muscle cells using a previously reported sphere-based culture. This protocol greatly improved sphere formation efficiency and stably induced the differentiation of myogenic cells from hiPS cells generated from both healthy donors and a patient with congenital myasthenic syndrome.

Risk of disseminated intravascular coagulation in patients with type 2 diabetes mellitus: retrospective cohort study.

Objectives: To determine quantitatively the association between type 2 diabetes mellitus (T2DM) and disseminated intravascular coagulation (DIC).

Design: Retrospective cohort study using a claims database.

Setting: Medical care institutions representing 9% of all secondary hospitals (acute care hospitals) in Japan.

External carotid artery stenting and superficial temporal artery to middle cerebral artery anastomosis for internal carotid artery occlusion with external carotid artery severe stenosis: case report.

Superficial temporal artery (STA) to middle cerebral artery (MCA) anastomosis may have inadequate effects in patients with internal carotid artery (ICA) occlusion and severe stenosis of the ipsilateral external carotid artery (ECA), because poor blood flow in the STA leads to insufficient flow to the MCA. In these patients, dilation of the stenotic ECA is required to improve the blood flow in the STA before STA-MCA anastomosis. A 71-year-old man presented with left hemiparesis and dysarthria.

Analysis of magnetic resonance imaging (MRI) morphometry and cerebral blood flow in patients with hypoxic-ischemic encephalopathy.

The aim of this study was to analyze magnetic resonance imaging (MRI) morphometry and cerebral blood flow (CBF) in patients with hypoxic-ischemic encephalopathy, and determine the viability of such measurements for predicting cerebral outcome. The subjects were 26 patients with histories of cardiopulmonary resuscitation who had subsequently developed encephalopathy. We examined the CBF using stable xenon-computed tomography (Xe-CT) and MR images in the subacute period (8-20 days).

Neuro-Behçet's disease manifesting as a neoplasm-like lesion--case report.

A 50-year-old man presented with neuro-Behçet's disease (NBD) manifesting as a large neoplasm-like lesion affecting the brainstem, basal ganglia, and white matter of the cerebral hemisphere. He had no history of disease except for psychoneurosis. On admission, neurological examination found left hemiparesis and dysarthria.