Aberrant evoked calcium signaling and nAChR cluster morphology in a D90A hiPSC-derived neuromuscular model.

Familial amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disorder that is due to mutations in one of several target genes, including . So far, clinical records, rodent studies, and models have yielded arguments for either a primary motor neuron disease, or a pleiotropic pathogenesis of ALS. While mouse models lack the human origin, models using human induced pluripotent stem cells (hiPSC) have been recently developed for addressing ALS pathogenesis.

Schwann cells in neuromuscular models.

Neuromuscular cell culture models are used to investigate synapse formation and function, as well as mechanisms of de-and regeneration in neuromuscular diseases. Recent developments including 3D culture technique and hiPSC technology have propelled their ability to complement insights from models. However, most cultures have not considered Schwann cells, the glial part of NMJs.

Nanoscale structural organization and stoichiometry of the budding yeast kinetochore.

Proper chromosome segregation is crucial for cell division. In eukaryotes, this is achieved by the kinetochore, an evolutionarily conserved multiprotein complex that physically links the DNA to spindle microtubules and takes an active role in monitoring and correcting erroneous spindle-chromosome attachments. Our mechanistic understanding of these functions and how they ensure an error-free outcome of mitosis is still limited, partly because we lack a complete understanding of the kinetochore structure in the cell.

Development and In Vitro Differentiation of Schwann Cells.

Schwann cells are glial cells of the peripheral nervous system. They exist in several subtypes and perform a variety of functions in nerves. Their derivation and culture in vitro are interesting for applications ranging from disease modeling to tissue engineering.

hiPSC-Derived Schwann Cells Influence Myogenic Differentiation in Neuromuscular Cocultures.

Motoneurons, skeletal muscle fibers, and Schwann cells form synapses, termed neuromuscular junctions (NMJs). These control voluntary body movement and are affected in numerous neuromuscular diseases. Therefore, a variety of NMJ in vitro models have been explored to enable mechanistic and pharmacological studies.

Effects of ASC Application on Endplate Regeneration Upon Glycerol-Induced Muscle Damage.

Amongst other approaches, adipose-derived stromal cells (ASCs) have recently been tested with respect to their regenerative capacity for treatment of neuromuscular disorders. While beneficial effects of ASCs on muscle recovery were observed previously, their impact on regeneration of neuromuscular junctions (NMJs) is unclear. Here, we used a murine glycerol damage model to study disruption and regeneration of NMJs and to evaluate the effects of systemic application of ASCs on muscle and NMJ recovery.

A Novel Optical Tissue Clearing Protocol for Mouse Skeletal Muscle to Visualize Endplates in Their Tissue Context.

Neuromuscular junctions (NMJs) mediate skeletal muscle contractions and play an important role in several neuromuscular disorders when their morphology and function are compromised. However, due to their small size and sparse distribution throughout the comparatively large, inherently opaque muscle tissue the analysis of NMJ morphology has been limited to teased fiber preparations, longitudinal muscle sections, and flat muscles. Consequently, whole mount analyses of NMJ morphology, numbers, their distribution, and assignment to a given muscle fiber have also been impossible to determine in muscle types that are frequently used in experimental paradigms.

Comprehensive strategy to reduce the incidence of lead dislodgement for cardiac implantable electronic devices.

Background: Lead dislodgement (LD) is a well-recognized complication during implantation of cardiac implantable electronic devices (CIEDs). An intraprocedural protocol, referred to as reduction of LD protocol, was developed to reduce the risk of LD.

Methods: The protocol involved (1) inserting a straight stylet down the right atrial lead and applying forward pressure while monitoring for fluoroscopic stability, (2) visualizing all leads during deep inspiration to determine if there is adequate lead redundancy, and (3) having the patient take a deep breath and cough while pacing just at capture threshold to assess for loss of capture in each lead.

Postnatal Development and Distribution of Sympathetic Innervation in Mouse Skeletal Muscle.

Vertebrate neuromuscular junctions (NMJs) have been conceived as tripartite synapses composed of motor neuron, Schwann cell, and muscle fiber. Recent work has shown the presence of sympathetic neurons in the immediate vicinity of NMJs and experimental and clinical findings suggest that this plays an eminent role in adult NMJ biology. The present study examined the postnatal development and distribution of sympathetic innervation in different muscles using immunofluorescence, confocal microscopy, and Western blot.