OBJECTIVE To investigate the genetic and physiologic basis of the neuromuscular symptoms of hypotonia-cystinuria syndrome (HCS) and isolated PREPL deficiency, and their response to therapy. METHODS We performed molecular genetic, histochemical, immunoblot, and ultrastructural studies, investigated neuromuscular transmission in vitro in a patient with isolated PREPL deficiency, and evaluated t...

The loose patch voltage clamp has been used to map Na current density along the length of snake and rat skeletal muscle fibers. Na currents have been recorded from (a) endplate membrane exposed by removal of the nerve terminal, (b) membrane near the endplate, (c) extrajunctional membrane far from both the endplate and the tendon, and (d) membrane near the tendon. Na current densities recorded d...

In the thoracolumbar region, between 7% and 30% of spinal fusion failures are at risk for pseudarthrosis. From a biomechanical perspective, the nonconformity of the intervertebral graft to the endplate surface could contribute to pseudarthrosis, given suboptimal stress distributions. The objective of this study was to quantify the effect of endplate-graft conformation on endplate stress distrib...

Methyl- and ethylguanidine block the endplate current in frog muscle. Both derivatives blocked inward-going endplate currents without affecting outward endplate currents. Repetitive stimulation that evoked several inward endplate currents enhanced the block, which suggests that these agents interact with open endplate channels. The relative conductance vs. potential curve exhibited a transition...

The loose patch voltage clamp has been used to map Na current density along the length of snake and rat skeletal muscle fibers . Na currents have been recorded from (a) endplate membrane exposed by removal of the nerve terminal, (b) membrane near the endplate, (c) extrajunctional membrane far from both the endplate and the tendon, and (d) membrane near the tendon . Na current densities recorded...

Cervical disc arthroplasty is a common method of treating cervical degenerative disease. However, the footprints of most prosthesis dimensions are obtained from data of Caucasian individuals. Besides, there is a large discrepancy between footprints of currently available cervical disc prostheses and anatomic dimensions of cervical endplates. We aimed to detail the three-dimensional (3D) anatomi...

OBJECTIVE Using alendronate after spinal fusion is a controversial issue due to the inhibition of osteoclast mediated bone resorption. In addition, there are an increasing number of reports that the endplate degeneration influences the lumbar spinal fusion. The object of this retrospective controlled study was to evaluate how the endplate degeneration and the bisphosphonate medication influence...