Spinal muscular atrophy (SMA) is caused by mutation of the Survival Motor Neurons 1 (SMN1) gene and is characterized by degeneration of spinal motor neurons. The severity of SMA is primarily influenced by the copy number of the SMN2 gene. Additional modifier genes that lie outside the SMA locus exist and one gene that could modify SMA is the Zinc Finger Protein (ZPR1) gene. To test the signific...

Challenges in purification and subsequent functionalization of membrane proteins often complicate their biochemical and biophysical characterization. Purification of membrane proteins generally involves replacing the lipids surrounding the protein with detergent molecules, which can affect protein structure and function. Recently, it was shown that styrene-maleic acid copolymers (SMA) can disso...

PURPOSE Lens epithelial cells transdifferentiate to myofibroblasts during the formation of anterior subcapsular cataracts and secondary cataracts. One of the defining characteristics of myofibroblasts is the expression of alpha-smooth muscle actin (alpha-SMA). This study investigated some of the factors that influence alpha-SMA expression in lens epithelial cells. METHODS Bovine, rabbit, and ...

We examined mechanotranscriptional regulation of the contractile gene, alpha-smooth muscle actin (SMA), in osteoblastic cells. Tensile forces were applied through collagen-coated magnetite beads to ROS17/2.8 cells. These cells were desmin-, vimentin+ and expressed low levels of SMA. After force application (480 piconewton/cell), SMA protein and mRNA were increased but beta-actin was unchanged. ...

Spinal muscular atrophy (SMA), a genetic neurodegenerative disorder, is caused by mutations or deletions in the survival of motor neuron 1 (SMN1) gene that result in SMN deficiency. SMN deficiency impairs microtubule networks in Smn-deficient cells and in SMA-like motor neuron cultures. Microtubule defects can be restored by knockdown of the stathmin gene (Stmn), which is upregulated in SMA. Ho...

In this paper, a new shape memory alloy (SMA) hybrid basalt fibre reinforced polymer (BFRP) composite laminate was fabricated and a new surface modification method with both silane coupling agent KH550 and Al₂O₃ nanoparticles was conducted to enhance the interface performance. The mechanical performance of BFRP composite laminates with and without SMA fibres and the influence of SMA surface mod...

OBJECTIVE To determine the compensatory mechanisms involved in the recovery of motor function following surgical lesions of the supplementary motor area (SMA) and their relation to the clinical characteristics of recovery. SUBJECTS AND METHODS Twelve patients were referred for surgery of low-grade gliomas located in the SMA, and compared to eight healthy controls using fMRI before and after s...

? Multiple Choice Questions Section The Neuroscience Journal introduces this new section on multiple choice questions as part of its commitment to continuous education and learning in Neurosciences. Experts in various neuroscience specialties are invited to participate with their knowledge and expertise in this section. Neurology, neurosurgery, and other board residents are encouraged to read t...

The loss and degeneration of spinal cord motor neurons result in muscle denervation in spinal muscular atrophy (SMA), but whether there are primary pathogenetic abnormalities of muscle in SMA is not known. We previously detected increased DNA fragmentation and downregulation of Bcl-2 and Bcl-X(L) expression but no morphological changes in spinal motor neurons of SMA fetuses. Here, we performed ...

Despite radical extension of surgical procedures, the cure rate of pancreatic head carcinoma patients still remains low. A cause of this concerns unsuccessful locoregional control, which may originate from a positive surgical margin near the superior mesenteric artery (SMA). However, no studies have examined invasion of pancreatic carcinoma around the SMA. En bloc resection of the head of the p...