A unique variant of lymphocytic choriomeningitis virus that induces pheromone binding protein MUP: Critical role for CTL.

Lymphocytic choriomeningitis virus (LCMV) WE variant 2.2 (v2.2) generated a high level of the major mouse urinary protein: MUP.

Flecainide ameliorates arrhythmogenicity through NCX flux in Andersen-Tawil syndrome-iPS cell-derived cardiomyocytes.

Andersen-Tawil syndrome (ATS) is a rare inherited channelopathy. The cardiac phenotype in ATS is typified by a prominent U wave and ventricular arrhythmia. An effective treatment for this disease remains to be established.

Epigenetic barrier against the propagation of fluctuating gene expression in embryonic stem cells.

The expression of pluripotency genes fluctuates in a population of embryonic stem (ES) cells and the fluctuations in the expression of some pluripotency genes correlate. However, no correlation in the fluctuation of Pou5f1, Zfp42, and Nanog expression was observed in ES cells. Correlation between Pou5f1 and Zfp42 fluctuations was demonstrated in ES cells containing a knockout in the NuRD component Mbd3.

Emerin plays a crucial role in nuclear invagination and in the nuclear calcium transient.

Alteration of the nuclear Ca transient is an early event in cardiac remodeling. Regulation of the nuclear Ca transient is partly independent of the cytosolic Ca transient in cardiomyocytes. One nuclear membrane protein, emerin, is encoded by EMD, and an EMD mutation causes Emery-Dreifuss muscular dystrophy (EDMD).

A Novel SCN5A Mutation Found in a Familial Case of Long QT Syndrome Complicated by Severe Left Ventricular Dysfunction.

A 16-year-old boy with long QT syndrome type 3 (LQT3) was admitted for decompensated heart failure resulting from dilated cardiomyopathy (DCM). His brother was also diagnosed with LQT3 and DCM. A comprehensive genetic analysis identified a novel SCN5A missense mutation-p.

Embryonic type Na channel β-subunit, SCN3B masks the disease phenotype of Brugada syndrome.

SCN5A is abundant in heart and has a major role in I. Loss-of-function mutation in SCN5A results in Brugada syndrome (BrS), which causes sudden death in adults. It remains unclear why disease phenotype does not manifest in the young even though mutated SCN5A is expressed in the young.

H1foo Has a Pivotal Role in Qualifying Induced Pluripotent Stem Cells.

Embryonic stem cells (ESCs) are a hallmark of ideal pluripotent stem cells. Epigenetic reprogramming of induced pluripotent stem cells (iPSCs) has not been fully accomplished. iPSC generation is similar to somatic cell nuclear transfer (SCNT) in oocytes, and this procedure can be used to generate ESCs (SCNT-ESCs), which suggests the contribution of oocyte-specific constituents.

Oral Nitrate Administration Ameliorates Cardiogenic Shock due to Eclipsed Mitral Regurgitation.

Eclipsed mitral regurgitation (MR) has been reported as transient massive functional MR caused by a sudden coaptation defect in the absence of left ventricular remodeling or epicardial coronary artery stenosis. Coronary spasm or microvascular dysfunction has been suggested to be associated with the pathogenesis. Here, we present a 68-year-old woman with eclipsed MR with cardiogenic shock ameliorated by nitrate.

Analysis of cardiomyocyte movement in the developing murine heart.

The precise assemblage of several types of cardiac precursors controls heart organogenesis. The cardiac precursors show dynamic movement during early development and then form the complicated heart structure. However, cardiomyocyte movements inside the newly organized mammalian heart remain unclear.

G-CSF supports long-term muscle regeneration in mouse models of muscular dystrophy.

Duchenne muscular dystrophy (DMD) is a chronic and life-threatening disease that is initially supported by muscle regeneration but eventually shows satellite cell exhaustion and muscular dysfunction. The life-long maintenance of skeletal muscle homoeostasis requires the satellite stem cell pool to be preserved. Asymmetric cell division plays a pivotal role in the maintenance of the satellite cell pool.

Impaired respiratory function in MELAS-induced pluripotent stem cells with high heteroplasmy levels.

Mitochondrial diseases are heterogeneous disorders, caused by mitochondrial dysfunction. Mitochondria are not regulated solely by nuclear genomic DNA but by mitochondrial DNA. It is difficult to develop effective therapies for mitochondrial disease because of the lack of mitochondrial disease models.

Patient-Specific Induced Pluripotent Stem Cell Models: Characterization of iPS Cell-Derived Cardiomyocytes.

Despite significant advances in medical treatment, cardiovascular disease is still a major cause of morbidity and mortality in advanced countries. To improve the outcome, the further promotion of basic cardiovascular science has a pivotal role for the developing novel therapeutic approach. However, due to the inaccessibility of human heart tissue, we couldn't obtain the sufficient amount of patient's heart tissues.

Endothelin-1 induces myofibrillar disarray and contractile vector variability in hypertrophic cardiomyopathy-induced pluripotent stem cell-derived cardiomyocytes.

Background: Despite the accumulating genetic and molecular investigations into hypertrophic cardiomyopathy (HCM), it remains unclear how this condition develops and worsens pathologically and clinically in terms of the genetic-environmental interactions. Establishing a human disease model for HCM would help to elucidate these disease mechanisms; however, cardiomyocytes from patients are not easily obtained for basic research. Patient-specific induced pluripotent stem cells (iPSCs) potentially hold much promise for deciphering the pathogenesis of HCM.

Thrombus in acute aortic dissection with atrial fibrillation: a treatment dilemma.

Type B acute aortic dissection (AAD) is often successfully managed with medical therapy, with a lower mortality rate, compared with type A AAD. Although the number of AAD patients complicated with atrial fibrillation (AF) has increased, reflecting an aging society, there have only been a few reports regarding the association of AAD and AF. Furthermore, there is no consensus on anticoagulation therapy in ADD patients complicated with AF, despite the importance of anticoagulation therapy in AF treatment.

Successful aortic root replacement and shunt closure in a case with rare coexistence of congenital cardiac malformations: bicuspid aortic valve with annuloaortic ectasia, single coronary artery, and patent foramen ovale.

This is the first report of rare simultaneous complication of three cardiac malformations: bicuspid aortic valve with annuloaortic ectasia, single coronary artery, and patent foramen ovale. We successfully operated to replace the aortic valve and ascending aorta, and to close the patent foramen ovale.

Generation and characterization of functional cardiomyocytes derived from human T cell-derived induced pluripotent stem cells.

Induced pluripotent stem cells (iPSCs) have been proposed as novel cell sources for genetic disease models and revolutionary clinical therapies. Accordingly, human iPSC-derived cardiomyocytes are potential cell sources for cardiomyocyte transplantation therapy. We previously developed a novel generation method for human peripheral T cell-derived iPSCs (TiPSCs) that uses a minimally invasive approach to obtain patient cells.

Distinct iPS Cells Show Different Cardiac Differentiation Efficiency.

Patient-specific induced pluripotent stem (iPS) cells can be generated by introducing transcription factors that are highly expressed in embryonic stem (ES) cells into somatic cells. This opens up new possibilities for cell transplantation-based regenerative medicine by overcoming the ethical issues and immunological problems associated with ES cells. Despite the development of various methods for the generation of iPS cells that have resulted in increased efficiency, safety, and general versatility, it remains unknown which types of iPS cells are suitable for clinical use.