BACKGROUND Because currently existing reinnervation methods result in poor functional recovery, there is a great need to develop new treatment strategies. OBJECTIVE To investigate the efficacy of our recently developed nerve-muscle-endplate band grafting (NMEG) technique for muscle reinnervation. METHODS Twenty-five adult rats were used. Sternohyoid (SH) and sternomastoid (SM) muscles serve...

We describe a severe congenital myasthenic syndrome (CMS) caused by two missense mutations in the gene encoding the muscle specific receptor tyrosine kinase (MUSK). The identified MUSK mutations M605I and A727V are both located in the kinase domain of MuSK. Intracellular microelectrode recordings and microscopy studies of the neuromuscular junction conducted in an anconeus muscle biopsy reveale...

We used the loose patch voltage clamp technique and rhodamine-conjugated alpha-bungarotoxin to study the regulation of Na channel (NaCh) and acetylcholine receptor (AChR) distribution on dissociated adult skeletal muscle fibers in culture. The aggregate of AChRs and NaChs normally found in the postsynaptic membrane of these cells gradually fragmented and dispersed from the synaptic region after...

The aim of this study was to explore the degree of degeneration of endplate chondrocytes in different tension regions induced by intermittent cyclic mechanical tension (ICMT) in vitro. Rat endplate chondrocytes were harvested and treated with 10% ICMT for 8 h/day with a frequency of 0.5 Hz. A cartilage degeneration model was induced using an FX‑5000T cell strain‑loading system. The experiment w...

Mutations in the plectin gene (PLEC1) cause epidermolysis bullosa simplex (EBS), which may associate with muscular dystrophy (EBS-MD) or pyloric atresia (EBS-PA). The association of EBS with congenital myasthenic syndrome (CMS) is also suspected to result from PLEC1 mutations. We report here a consanguineous patient with EBS and CMS for whom mutational analysis of PLEC1 revealed a homozygous 36...

The objective of this study was to systematically characterize motor units (MUs) of the musculature of the lower face. MU endplate positions and principal muscle fiber orientations relative to facial landmarks were identified. This was done by the analysis of motor unit action potentials (MUAPs) in the surface electromyogram. Thirteen specially trained, healthy subjects performed selective cont...

Bone is a dynamic structure and is known to respond to changes in the load over time, in accordance with Wolff’s law. It states that the bone changes its shape and internal architecture in response to stresses acting on it [1]. Therefore, any structural changes in the spine may lead to bone remodeling due to changes in the optimal stress pattern. The changes in apparent density and thickness of...

We have developed a technique, called synaptic reconstruction, that permits nerve terminals of living vertebrate neuromuscular junctions (NMJs) to be isolated and then manually recombined with vacant endplate sites to form functional synapses. By reconstructing NMJs with various combinations of pre- and postsynaptic partners, or with varying degrees of pre- to postsynaptic alignment, the functi...