Nuclear localization of a novel calpain-2 mediated junctophilin-2 C-terminal cleavage peptide promotes cardiomyocyte remodeling.

Heart failure (HF) is a leading cause of morbidity and mortality worldwide. Patients with HF exhibit a loss of junctophilin-2 (JPH2), a structural protein critical in forming junctional membrane complexes in which excitation-contraction takes place. Several mechanisms have been proposed to mediate the loss of JPH2, one being cleavage by the calcium-dependent protease calpain.

Effects of Korsakoff Amnesia on performance and symptom validity testing.

Performance validity tests (PVTs) and Symptom validity tests (SVTs) are developed to identify people that present false or exaggerated symptoms. Although a key factor of both types of tests includes relative insensitivity to cognitive disorders, the direct effects of amnesia have been poorly studied. Therefore, a sample of 20 patients diagnosed with Korsakoff Amnesia (KA) through neuropsychological assessment and 20 healthy comparisons (HC) were administered the Test of Memory Malingering (TOMM), the Structured Inventory of Malingered Symptomatology (SIMS), and the newly developed Visual Association Test - Extended (VAT-E).

The MEF2 transcriptional target DMPK induces loss of sarcomere structure and cardiomyopathy.

Aims: The pathology of heart failure is characterized by poorly contracting and dilated ventricles. At the cellular level, this is associated with lengthening of individual cardiomyocytes and loss of sarcomeres. While it is known that the transcription factor myocyte enhancer factor-2 (MEF2) is involved in this cardiomyocyte remodelling, the underlying mechanism remains to be elucidated.

The RNA-binding protein Rbm38 is dispensable during pressure overload-induced cardiac remodeling in mice.

The importance of tightly controlled alternative pre-mRNA splicing in the heart is emerging. The RNA binding protein Rbm24 has recently been identified as a pivotal cardiac splice factor, which governs sarcomerogenesis in the heart by controlling the expression of alternative protein isoforms. Rbm38, a homolog of Rbm24, has also been implicated in RNA processes such as RNA splicing, RNA stability and RNA translation, but its function in the heart is currently unknown.

A mutation in the glutamate-rich region of RNA-binding motif protein 20 causes dilated cardiomyopathy through missplicing of titin and impaired Frank-Starling mechanism.

Aim: Mutations in the RS-domain of RNA-binding motif protein 20 (RBM20) have recently been identified to segregate with aggressive forms of familial dilated cardiomyopathy (DCM). Loss of RBM20 in rats results in missplicing of the sarcomeric gene titin (TTN). The functional and physiological consequences of RBM20 mutations outside the mutational hotspot of RBM20 have not been explored to date.