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...

هدف: آینده­ نگری مجموعه ­ای از توانایی­ های شناختی است. که شامل پیش­ بینی حالت­ ممکن آینده و نیازهای خود این مهارت برای برنامه­ ریزی، غلبه بر موانع رسیدن به موفقیت افراد کمک می­کند. یکی جدیدترین ابزارهای سنجش توانایی تفکر آینده­نگر در کودکان، مقیاس کودکان (مازاچووسکای مهی، 2020) است ویژگی­ روان­سنجی آن استفاده جمعیت ایرانی مورد مطالعه قرار نگرفته روش: روش پژوهش حاضر توصیفی مقطعی بود. تعداد 200 ...

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...

Spinal muscular atrophy (SMA) is a genetic disease characterized by atrophy of muscle and loss of spinal motor neurons. SMA is caused by deletion or mutation of the survival motor neuron 1 (SMN1) gene, and the nearly identical SMN2 gene fails to generate adequate levels of functional SMN protein due to a splicing defect. Currently, several therapeutics targeted to increase SMN protein are in cl...

Spinal muscular atrophy (SMA) is a common autosomal recessive neuromuscular disorder caused by mutations in the survival motor neuron (SMN1) gene, affecting approximately 1 in 10,000 live births. The homozygous absence of SMN1 exon 7 has been observed in the majority of patients and is being utilized as a reliable and sensitive SMA diagnostic test. Treatment and prevention of SMA are complement...