A Systemically Administered Unconjugated Antisense Oligonucleotide Targeting DUX4 Improves Muscular Injury and Motor Function in FSHD Model Mice.

Facioscapulohumeral muscular dystrophy (FSHD), one of the most common muscular dystrophies, is caused by an abnormal expression of the DUX4 gene in skeletal muscles, resulting in muscle weakness. In this study, we investigated MT-DUX4-ASO, a novel gapmer antisense oligonucleotide (ASO). MT-DUX4-ASO decreased the expression of DUX4 and its target genes in FSHD patient-derived myoblasts.

Flying characterization of colliding partners by hyperfine splitting frequency of neutral paramagnetic atoms.

The pseudopotentials and dispersion potentials are applied to a theoretical study of the hyperfine splitting frequencies of the ground-state paramagnetic hydrogen (H) and alkali-metal (Li, Na, K, Rb, and Cs) atoms in noble gases (He, Ne, Ar, Kr, and Xe). Using classical turning points for statistical averages, we find that numerical calculations based on second-order perturbation theory fit the measured frequency shifts well over a wide temperature range. The characteristic energy, pseudopotential height, and electric-dipole polarizability allow us to consistently determine the van der Waals radii and electron scattering lengths of noble-gas atoms.

Pseudopotential analysis on hyperfine splitting frequency shift of alkali-metal atoms in noble gases, revisited.

Theoretical pseudopotentials and dispersion potentials are used to study ground-state hyperfine splitting frequencies of alkali-metal atoms (Li, Na, K, Rb, and Cs) in noble gases (He, Ne, Ar, Kr, and Xe) in all combinations. With a single fitting parameter, calculations based on first-order perturbation theory qualitatively present each temperature dependence of the measured frequency shift. With this parameter and excitation energies of alkali-metal and noble-gas atoms, the hyperfine splitting frequency of alkali-metal atoms is suitable for investigating the properties of noble-gas atoms, such as the s-wave scattering length of electrons, the electric-dipole polarizability, and the van der Waals radius.

[Cyclic thrombocytopenia diagnosed by periodic platelet count measurements].

Cyclic thrombocytopenia (CTP) is characterized by periodic platelet count fluctuations and is commonly misdiagnosed as immune thrombocytopenia (ITP) because of their similar clinical characteristics. Here, we present the case of a 74-year-old man with CTP diagnosed by weekly platelet count measurements. The patient initially developed mild bleeding symptoms with a platelet count of 0.

Noble-gas atoms characterized by hyperfine frequency shift of lithium atom.

We report an experimental and theoretical study on the shift of the hyperfine splitting frequency of ground-state Li atoms in noble gases, He, Ne, Ar, and Xe. The frequency shift is due to the change in the electron-spin density at the Li nuclei induced by collisions to the noble-gas atoms. The electron density is calculated along the interatomic distance in a pseudopotential and a dispersion potential.

Nephronectin influences EAE development by regulating the Th17/Treg balance via reactive oxygen species.

Blood levels of the extracellular matrix protein nephronectin (Npnt), a protein critical for kidney development, are elevated in autoimmune experimental autoimmune encephalitis (EAE) mice, which are a model for multiple sclerosis. We found here that treatment with anti-Npnt antibody directed against the αβ integrin-binding site (Npnt-blocking antibody) inhibits EAE development. The selenium transporter selenoprotein P (SeP) was identified as a novel Npnt-binding partner.

Antibodies against nephronectin ameliorate anti-type II collagen-induced arthritis in mice.

The extracellular matrix protein nephronectin (Npnt) is known to be critical for kidney development, but its function in inflammatory diseases is unknown. Here, we developed a new enzyme-linked immunosorbent assay system to detect Npnt in various autoimmune diseases, which revealed that plasma Npnt levels are increased in various mouse autoimmune models. We also report that antibodies against the α8β1 integrin-binding region of Npnt protect mice from anti-type II collagen-induced arthritis, suggesting that Npnt may be a potential therapeutic target molecule for the prevention of autoimmune arthritis.

Natriuretic peptide signaling is involved in the expression of oxidative metabolism-related and muscle fiber constitutive genes in the gastrocnemius muscle.

Natriuretic peptides regulate cyclic guanosine monophosphate (cGMP) levels via their receptors and have various physiological effects. Natriuretic peptide receptor C (NPR-C) increases cGMP signaling by functioning as a clearance receptor. We analyzed the role of natriuretic peptides in the skeletal muscle, which increases in mass with bone elongation, of NPR-C- mice.

Involvement of natriuretic peptide system in C2C12 myocytes.

The natriuretic peptide system, a key regulator of cGMP signaling, comprises three types of natriuretic peptides, osteocrin/musclin (OSTN), and their natriuretic peptide receptors. Although this system plays important roles in many organs, its physiological roles in skeletal muscle have not been clearly described. In the present study, we investigated the role of the natriuretic peptide system in C2C12 myocytes.

Morphology-Based Analysis of Myoblasts for Prediction of Myotube Formation.

The development of new drugs depends on the efficiency of drug screening. Phenotype-based screening has attracted interest due to its considerable potency for the discovery of first-in-class drugs. In general, fluorescently labeled imagery is the leading technique for phenotype-based screening; however, there are growing requirements to understand total culture profiles, which are unclear after end-point assays.

Spectroscopic study of a diffusion-bonded sapphire cell for hot metal vapors.

Characteristics of a diffusion-bonded sapphire cell for optical experiments with hot metal vapors were investigated. The sapphire cell consisted of sapphire-crystal plates and a borosilicate-glass tube, which were bonded to each other by diffusion bonding without any binders or glues. The glass tube was attached to a vacuum manifold using the standard method applied in glass processing, filled with a small amount of Rb metal by chasing with a torch, and then sealed.

Analysis of gastrointestinal metastasis of primary lung cancer: Clinical characteristics and prognosis.

The prevalence of gastrointestinal metastasis of lung cancer is low. The aim of the present study was to analyze the frequency and clinical characteristics of metastases to the gastrointestinal tract by retrospectively assessing the clinical records of 2,066 patients with lung cancer. A total of 7 patients (0.

Repeated Localized Treatment for Endobronchial Metastasis of Thymic Carcinoma.

Background: Endobronchial metastases derived from nonpulmonary tumors are uncommon, although a variety of malignant tumors have been reported to be associated with endobronchial metastasis. We herein report a case of repeated bronchoscopic resection of endobronchial metastasis of a thymic carcinoma.

Case Report: A 59-year-old woman was diagnosed with primary thymic carcinoma, Masaoka stage IVA, in May 2009.

A case of synchronous double primary lung cancer presenting with pleomorphic carcinoma and adenocarcinoma.

Background: Recently, synchronous multiple lung cancer (SMPLC) has sometimes been detected as a result of improved radiological imaging, although the occurrence of SPMLC is still rare. To the best of our knowledge, there have been no reported cases of with synchronous double primary lung cancer presenting with pleomorphic carcinoma and adenocarcinoma. We herein report such a case.

Hyperpolarized cesium ions doped in a glass material.

Hyperpolarized (HP) Cs nuclear magnetic resonance signals were measured from borosilicate glass cell walls during optical pumping of cesium vapor at high magnetic field (9.4T). Significant signal enhancements were observed when additional heating of the cell wall was provided by intense but non-resonant laser irradiation, with integrated HP Cs NMR signals and line widths varying as a function of heating laser power (and hence glass temperature).

Long-term survival after surgical treatment of metachronous bilateral adrenal metastases of non-small cell lung carcinoma.

Background Although resection of the metastases is the treatment of choice for unilateral solitary adrenal metastasis of non-small cell lung carcinoma (NSCLC), the surgical treatment for bilateral adrenal metastases is quite rare, likely due to the coexistence of multiple synchronous metastases at other sites and/or primary adrenal insufficiency following bilateral adrenalectomy. We herein report a rare case of asynchronous metastasis of NSCLC to the bilateral adrenal glands with long-term survival after bilateral adrenalectomy. Case Report A 70-year-old male underwent right upper lobectomy for lung adenocarcinomaT2aN2M0, stageIIIA following induction chemotherapy.

An analysis of and new risk factors for reexpansion pulmonary edema following spontaneous pneumothorax.

Background: The major risk factor for reexpansion pulmonary edema (RPE) following the treatment of spontaneous pneumothorax is thought to be chronic lung collapse. However, a long-term collapsed lung does not always cause RPE. The purpose of this study was to define other risk factors for RPE among patients undergoing drainage for the treatment of spontaneous pneumothorax.

Utility of the serum ProGRP level for follow-up of pulmonary carcinoid tumors.

Patient: Female, 67.

Final Diagnosis: Pulmonary carcinoid tumor.

Symptoms: Abnormal shadow on chest X-ray.

Lung cancer mimicking lung abscess formation on CT images.

Patient: Male, 64 FINAL DIAGNOSIS: Lung pleomorphic carcinoma Symptoms: Cough • fever

Medication: - Clinical Procedure: - Specialty: Oncology.

Objective: Unusual clinical course.

Background: The diagnosis of lung cancer is often made based on computed tomography (CT) image findings if it cannot be confirmed on pathological examinations, such as bronchoscopy.

Salvage operation for late recurrence after stereotactic body radiotherapy for lung cancer: two patients with no viable cancer cells.

We report two patients who underwent salvage lung resection for suspected local recurrence on computed tomography image findings after stereotactic body radiotherapy; however, the pathologic findings indicated no viable tumor cells. Distinguishing between posttreatment changes and tumor recurrence after stereotactic body radiotherapy on the image findings is difficult; therefore, the determination of surgical indications requires comprehensive evaluations.

Right lower lobectomy with middle lobe preservation after right upper lobectomy in lung cancer of the right lower lobe.

A 73-year-old woman who underwent right upper lobectomy for tuberculosis 40 years earlier was diagnosed with adenocarcinoma, clinical stage IIA (T2bN0M0), in the right lower lobe of her lung. A lower lobectomy with preservation of the middle lobe was performed. The patient had an uneventful recovery.

Expression of a truncated form of the endoplasmic reticulum chaperone protein, σ1 receptor, promotes mitochondrial energy depletion and apoptosis.

The σ1 receptor (σ(1)R) regulates endoplasmic reticulum (ER)/mitochondrial interorganellar Ca(2+) mobilization through the inositol 1,4,5-trisphosphate receptor (IP(3)R). Here, we observed that expression of a novel splice variant of σ(1)R, termed short form σ(1)R (σ(1)SR), has a detrimental effect on mitochondrial energy production and cell survival. σ(1)SR mRNA lacks 47 ribonucleotides encoding exon 2, resulting in a frameshift and formation of a truncated receptor.

Efficacy and tolerance of gastrostomy feeding in Japanese muscular dystrophy patients.

Although muscular dystrophy patients often have feeding difficulty and need long-term enteral nutrition, only a few reports have described gastrostomy feeding in these patients. This study was designed to evaluate the efficacy and tolerance of gastrostomy feeding in patients with muscular dystrophy. We performed a retrospective, multicenter study on 144 patients with muscular dystrophy who received gastrostomy feeding between 2007 and 2009 in 25 neuromuscular centers in Japan.

A 10-b 50-MS/s 820- μW SAR ADC With On-Chip Digital Calibration.

This 10-b 50-MSamples/s SAR analog-to-digital converter (ADC) features on-chip digital calibration techniques, comparator offset cancellation, a capacitor digital-to-analog converter (CDAC) linearity calibration, and internal clock control to compensate for PVT variations. A split-CDAC reduces the exponential increase in the number of unit capacitors needed and enables the input load capacitance to be as small as the kT/C noise restriction. The prototype fabricated in 65 nm 1P7M complementary metal-oxide semiconductor with MIM capacitor achieves 56.

Simultaneous occurrence of partial anomalous pulmonary venous return and major bronchial anomaly: computed tomography findings in 5 adult patients.

Objective: The objective of this study was to describe the computed tomography (CT) findings of 5 adult patients with a combination of partial anomalous pulmonary venous return (PAPVR) and major bronchial anomaly.

Methods: A computerized search of the radiological database records of 4886 consecutive patients who underwent chest CT from April 2005 to May 2007 described 2 patients with a combination of PAPVR and tracheal bronchus (TB). Three additional patients with a combination of PAPVR and TB or accessory cardiac bronchus (ACB) were obtained by reviewing the CT images of the patients initially diagnosed as having PAPVR, TB, or ACB at our institution during the same period.