نتایج جستجو برای: آتروفی عضلانی نخاعی sma
تعداد نتایج: 25982 فیلتر نتایج به سال:
Expression of alpha-smooth muscle actin (alpha-SMA) defines the phenotype of activated (myofibroblastic) hepatic stellate cells. These cells, but not quiescent stellate cells, have a high level of alpha-SMA and c-Myb expression, as well as increased c-Myb-binding activities to the proximal alpha-SMA E box. Therefore, we analyzed the role of c-Myb in alpha-SMA transcription and stellate cell act...
Spinal muscular atrophy (SMA) is an autosomal recessive disorder, which is the leading genetic cause of infantile death. SMA is the most common inherited motor neuron disease and occurs in approximately 1:6000 live births. The gene responsible for SMA is called Survival Motor Neuron-1 (SMN1). Interestingly, a human-specific copy gene is present on the same region of chromosome 5q, called SMN2. ...
The autosomal recessive neurodegenerative disease spinal muscular atrophy (SMA) results from low levels of survival motor neuron (SMN) protein; however, it is unclear how reduced SMN promotes SMA development. Here, we determined that ubiquitin-dependent pathways regulate neuromuscular pathology in SMA. Using mouse models of SMA, we observed widespread perturbations in ubiquitin homeostasis, inc...
Most patients with spinal muscular atrophy (SMA) have been reported to show homozygous deletion of the gene responsible for SMA, SMN1. However, whether SMA patients homozygous for the SMN1 deletion exist in Southeast Asian countries, including Vietnam, remains to be determined, because molecular genetic analyses of SMA patients from these countries have not been reported. In this preliminary st...
Human fibrotic bone marrow (BM) stroma has been shown to contain alpha-smooth muscle actin (alpha-SMA)-positive cells. These closely resemble myofibroblasts that were described in other fibrotic tissues. We studied the expression of alpha-SMA in a series of murine BM-derived stromal cell lines to investigate the cellular origin and functional significance of myofibroblast-like cells in hematopo...
The ability to assess temporal structure is crucial in order to adapt to an ever-changing environment. Increasing evidence suggests that the supplementary motor area (SMA) is involved in both sensory and sensorimotor processing of temporal structure. However, it is not entirely clear whether the structural differentiation of the SMA translates into functional specialization, and how the SMA rel...
Spinal muscular atrophy (SMA) is a neuromuscular disorder characterized by motor neuron degeneration, although defects in multiple cell types and tissues have also been implicated. Three independent laboratories recently identified immune organ defects in SMA. We therefore propose a novel pathogenic mechanism contributory to SMA, resulting in higher susceptibility to infection and exacerbated d...
آیا پرداختن به ورزش و فعالیتهای منظم تربیت بدنی می تواند تغییراتی مطلوب در ساختار بدنی و فیزیولوژیکی افراد ضایع نخاعی مانند سایر افراد سالم به وجود آورد؟ آیا افراد ضایعه نخاعی که به طور منظم در فعالیتهای ورزشی شرکت می کنند از نظر جسمانی ( عضلات قفسه سینه ) با همردیفان خود که ورزش نمی کنند تفاوت دارند؟ آیا افراد ضایعه نخاعی ورزشکار و غیرورزشکار در رشته های بسکتبال با ویلچر و پینگ پنگ دارای دامنه...
In the past few years, extensive improvements have been accomplished in reinforcing the structures through using shape memory alloys (SMAs). These materials absorb or dissipate energy through establishing a reversible hysteresis loop during a cyclic mechanical loading. This unique characteristic of the SMAs has made them appropriate for sensing, actuation, absorbing the impact energy, and vibra...
Background Tuberous Sclerosis Complex (TSC), and Spinal Muscular Atrophy (SMA) are two inherited disorders while they are genetically independent. TSC is characterized by the formation of multiple hamartomas in nearly all organs. SMA is a destructive neurological disorder leading to progressive muscular weakness and atrophy. Case Presentation</e...
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