spinal muscular atrophy (sma) is an autosomal recessive neuromuscular disorder caused by homozygous deletion of the survival motor neuron gene 1 (smn1) in more than 90% of patients. according to the age of onset and severity of the disease, sma is classified into three groups: type i (severe), type ii (intermediate) and type iii (mild). as reported, the smn2 gene, centromeric copy gene, showed ...

We report the first asymmetric sulfa-Michael addition (SMA) reactions using a chiralN-heterocyclic carbene (NHC) as a non-covalent organocatalyst. We demonstrate that a triazolium salt derived NHC functions as an excellent Brønsted base to promote enantioselective carbon–sulfur bond formation. The reaction is applicable to a wide range of thiols and electrophilic olefins. Notably, quaternary ch...

A 28-year-old patient with pharmacoresistant non-lesional right frontal epilepsy underwent extra-operative intracranial EEG recordings and electrical cortical stimulation (ECS) to map eloquent cortex. Right supplementary motor area (SMA) ECS induced a brief seizure with habitual symptoms involving neck tingling followed by asymmetric tonic posturing. An additional feature was neck atonia. Durin...

t his paper investigates the asymmetric behavior of inflation. we use logistic smooth transition autoregressive (lstar) model to characterize the regime-switching behavior of iran’s monthly inflation during the period may 1990 to december 2013. we find that there is a triple relationship between the inflation level, its fluctuations and persistence. the findings imply that the behavior of infla...

In this paper, a modular microactuator based on shape memory alloy (SMA) actuation to drive the joints of a microgripper is described. Its design is a combination of two SMA actuators installed on the links of a special mechanism : one of them drives the rotation of the joint and the other one produces the necessary force to manipulate objects. An accurate model of the thermo-mechanical behavio...

Many models simulating the behavior of Shape Memory Alloys (SMA) exist nowadays, in the one or three dimensional situation. In most of the cases they are not able to describe the non isothermal behavior of SMA due to the thermomechanical loadings. Indeed, the influence of the stress on the product phase leads to an orientation of the variants, when the temperature creates a self-accomodating ma...

Motor control is integral to all types of human behavior, and the dorsal Anterior Cingulate Cortex (dACC) is thought to play an important role in the brain network underlying motor control. Yet the role of the dACC in motor control is under-characterized. Here we aimed to characterize the dACC's role in adolescent brain network interactions during a simple motor control task involving visually ...

 Background: Spinal muscular atrophy (SMA) is the second most common lethal autosomal recessive disease. It is a neuromuscular disorder caused by degenerative of lower motor neurons and occasionally bulbar neurons leading to progressive limb paralysis and muscular atrophy. The SMN1 gene is recognized as a SMA causing gene while NAIP has been characterized as a modifying factor for the clinical ...

in this paper, seismic behavior of the existing buildings equipped by friction dampers is studied. seismic performance of6-story, 9-story and 12-story steel buildings with damper and without damper were studied. the finite element modeling technique (sap2000 software) is used for analysis. time history analyzing was done to achieve this purpose. for nonlinear dynamic analysis, the responses of ...