Spinal muscular atrophy (SMA) is a frequent neuromuscular disorder caused by motoneuronal apoptosis, as a result of SMN (Survival Motor Neuron) protein deficiency. Although the SMA determining gene was identified, the molecular mechanism of the disease is not clearly understood, due to the heterogeneity of clinical manifestations. Trying to complete the molecular describing SMA picture, by iden...

Notch signaling regulates smooth muscle cell phenotype and is critical for vascular development. One Notch target is smooth muscle alpha-actin (SMA), a differentiated smooth muscle cell marker. The Notch intracellular domain (NotchICD) forms a complex with CBF-1 (C-promoter-binding factor-1) and directly induces SMA expression. Using primary human smooth muscle cells, we show that expression of...

OBJECTIVE This study, which aimed to confirm or invalidate the somatotopic organization of the supplementary motor area (SMA), correlates the pattern of clinical symptoms observed after SMA removal with the extent of resection. METHODS Eleven patients with medial precentral glioma underwent partial or complete tumoral resection of the SMA. Seven patients underwent preoperative functional magn...

Spinal muscular atrophy (SMA) is an autosomal recessive disease characterized by degeneration of the anterior horn cells of the spinal cord, leading to muscular paralysis with muscular atrophy. No effective treatment of this disorder is presently available. Studies of the correlation between disease severity and the amount of survival motor neuron (SMN) protein have shown an inverse relationshi...

Proximal spinal muscular atrophy (SMA) is an early onset, autosomal recessive motor neuron disease caused by loss of or mutation in SMN1 (survival motor neuron 1). Despite understanding the genetic basis underlying this disease, it is still not known why motor neurons (MNs) are selectively affected by the loss of the ubiquitously expressed SMN protein. Using a mouse embryonic stem cell (mESC) m...

Guaranteeing the use of Shape Memory Alloys (SMA) in mitigation of extreme load effects requires a deep study of the SMA behavior according to the specific requirements of the applications in damping. The damper was defined according the expected requirements (length of SMA and number of SMA wires). It is applied to two types of alloys (CuAlBe and NiTi) in the diagonals of a realistic steel por...

Since the 1960s, Stone Mastic Asphalt (SMA) pavement surfaces have been used successfully in Germany on heavily trafficked roads. Because of its excellent performance characteristics, road authorities in Germany as well as major European Countries quickly adopted SMA as a standard wearing course. As a consequence of an asphalt study tour in Europe of an American delegation in 1990, SMA test sec...

Superelastic Shape Memory Alloy (SMA) is accepted when it used as connection in steel structures. The seismic behaviour of steel frames with SMA is being assessed in this study. Three eightstorey steel frames with different SMA systems are suggested, the first one of which is braced with diagonal bracing system, the second one is braced with nee bracing system while the last one is which the SM...

Two sibs affected by the severe neonatal form of spinal muscular atrophy (SMA) with diaphragmatic paralysis are described. The two sibs were discordant for the haplotypes determined by DNA markers flanking the SMA locus. This supports non-linkage of SMA to chromosome 5 in this family and indicates that the uncommon SMA type I variant associated with early onset respiratory failure maps outside ...

To evaluate whether alpha-smooth muscle actin (alpha-SMA) plays a role in fibroblast contractility, we first compared the contractile activity of rat subcutaneous fibroblasts (SCFs), expressing low levels of alpha-SMA, with that of lung fibroblasts (LFs), expressing high levels of alpha-SMA, with the use of silicone substrates of different stiffness degrees. On medium stiffness substrates the p...