OBJECTIVE To investigate the potential association of plastin 3 (PLS3) expression levels in the blood with disease severity in spinal muscular atrophy (SMA). DESIGN Measurement of PLS3 messenger RNA levels in the blood of patients with types I, II, and III SMA. SETTING Pediatric Neuromuscular Clinical Research Network SMA Natural History study. PARTICIPANTS A cohort of 88 patients of both...

OBJECTIVE To test whether therapeutic unilateral deep brain stimulation (DBS) of the subthalamic nucleus (STN) in patients with Parkinson disease (PD) leads to normalization in the pattern of brain activation during movement execution and control of movement extent. METHODS Six patients with PD were imaged off medication by PET during performance of a visually guided tracking task with the DB...

The caudal cingulate motor area (CMAc) and the supplementary motor area (SMA) play important roles in movement execution. The present study examined the neural mechanisms underlying these roles by investigating local field potentials (LFPs) from these areas while monkeys pressed buttons with either their left or right hand. During hand movement, power increases in the high-gamma (80-120 Hz) and...

Spinal muscular atrophy (SMA), characterized by specific degeneration of spinal motor neurons, is caused by mutations in the survival motor neuron 1 (SMN1) gene and subsequent decreased levels of functional SMN. How the deficiency of SMN, a ubiquitously expressed protein, leads to spinal motor neuron-specific degeneration in SMA patients remains unknown. In this study, we examined the role of S...

Spinal muscular atrophy (SMA), characterized by specific degeneration of spinal motor neurons, is caused by mutations in the survival motor neuron 1 (SMN1) gene and subsequent decreased levels of functional SMN. How the deficiency of SMN, a ubiquitously expressed protein, leads to spinal motor neuron-specific degeneration in SMA patients remains unknown. In this study, we examined the role of S...

We analyzed neuronal activity in the supplementary eye field (SEF), supplementary motor area (SMA), and presupplementary motor area (pre-SMA) during the performance of three motor tasks: capturing a visual target with a saccade, reaching one arm to a target while gazing at a visual fixation point, or capturing a target with a saccade and arm-reach together. Our data demonstrated that each area ...

Total DNA was isolated from swine feces and a swine waste storage pit and used as templates for PCR amplification of archaeal 16 rDNA using specific primers. Only the sample from the center of the waste pit produced a PCR product. DNA sequence analyses of random clones demonstrated a variety of methanogenic archaea. Six groups of sequences were identified, including those similar to Methanobrev...

PeiW (UniProtKB Q7LYX0) and PeiP (UniProtKB Q77WJ4) are the two major pseudomurein endoisopeptidases (Pei) that are known to cleave pseudomurein cell-wall sacculi of the members of the methanogenic orders Methanobacteriales and Methanopyrales. Both enzymes, originating from prophages specific for some methanogenic archaeal species, hydrolyze the ϵ(Ala)-Lys bond of the peptide linker between adj...