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Laminin α2 chain-deficient muscular dystrophy, or LAMA2-CMD, is a very severe disease caused by mutations in the LAMA2 gene. Skeletal muscle is the most affected tissue, with patients presenting symptoms such as hypotonia at birth, joint contractures and progressive muscle wasting. Changes in the central nervous system include white matter abnormalities, delayed motor milestones and compromised ac
A price can be set in different ways for a manufactured component, most commonly based on estimated manufacturing costs, market intelligence, and market adopted pricing or customer value. Pricing is one of the key factors when selecting subcontractors in the manufacturing sector. As a subcontractor, there are certain risks connected with pricing of a product, too low pricing leads to losses, where
In the past decade, high throughput screening (HTS) has changed the way biochemical assays are performed, but manipulation and mechanical measurement of micro- and nanoscale systems have not benefited from this trend. Techniques using microbeads (particles approximately 0.1-10 mum) show promise for enabling high throughput mechanical measurements of microscopic systems. We demonstrate instrumentat
The tremendous development in the past ten last years of the Internet and ICT at large (whether it be in general technologies like semantic modeling, knowledge mining, RFID or mobile technologies, or domain-oriented ones like e-commerce, collaborative spaces, digital mock-ups, etc.) has opened a large spectrum of potential applica-tions of ICT in the Construction sector. The real adaptation and de
Periodic boundary conditions (PBC) are well suited to describe repetitive structures in space, yet flawed for isotropic setups in which it is commonly used. We present an approach based on exploiting the congruency of a unit cell which combines the periodic nature of PBC with isotropy of an alternating cell. By applying these isotropic periodic boundary conditions (IPBC) to the standard Ewald summ
Head turning experiments are widely used to test the cognition of both human infants and non-human animal species. Monitoring head turns allows researchers to non-invasively assess attention to acoustic or visual stimuli. In the majority of head turning experiments, the head direction analyses have been accomplished manually, which is extremely labor intensive and can be affected by subjectivity o
Membrane applications have established themselves in both the production and water loop of the starch-based sweetener industry. This presentation will give an overview of membrane opportunities in the starch-based sweetener industry is given. In the first part of this paper, the membrane opportunities in the production loop of starch-based sweeteners will be generally discussed and application stu
We have fabricated nanometer-sized interdigitated electrode patterns using electron beam lithography and liftoff techniques. The aim of the investigation was to find out whether the dimensions (i.e., the electrode separations) of the pattern would affect the admittance signal of the biomolecules in between the electrodes. Since the admittance signal scales with the geometrical factor A/d, where A
IntroductionMembrane processes can be either used in direct competition with conventional separation processes or in combination with these to cover a wider separation range. The focus of this work is on the applications in an apple juice plant using membrane processes in combination with conventional separation processes, i.e. separators and evaporators.Traditional Apple Juice Plant The traditio
Heat supply bottlenecks are common in district heating (DH) networks and makes the DH network work in a non-optimal way, why it is beneficial to address them. The purpose of this paper is thus to investigate risks, opportunities, added values and issues coupled to various bottleneck measures. This is made by in depth interviews with DH companies and through DH network simulations. The results show
We present two new data structures for computing values of an n-variate polynomial P of degree at most d over a finite field of q elements. Assuming that d divides q- 1 , our first data structure relies on (d+ 1) n + 2 tabulated values of P to produce the value of P at any of the qn points using O(nqd2) arithmetic operations in the finite fie
In this article, we describe a new command, xtbcfe, that performs the iterative bootstrap-based bias correction for the fixed-effects estimator in dynamic panels proposed by Everaert and Pozzi (2007, Journal of Economic Dynamics and Control 31: 1160–1184). We first simplify the core of their algorithm by using the invariance principle and subsequently extend it to allow for unbalanced and higher o
Pituitary adenylate cyclase activating polypeptide-38 (PACAP38) is a widely distributed neuropeptide involved in neuroprotection, neurodevelopment, nociception and inflammation. Moreover, PACAP38 is a potent inducer of migraine-like attacks, but the mechanism behind this has not been fully elucidated.Migraine is a neurovascular disorder, recognized as the second most disabling disease. Nevertheles
Innovation in retailing is under-investigated in academia and yet highly relevant in practice given the current changes in the retail landscape. Although retailing is often characterised by a dynamic and highly competitive environment, retail organizations are not often considered as “innovative,” at least when compared with manufacturing industries or when using existing innovation frameworks in
Remote sensing is a key tool for precision agriculture applications as it is capable of capturing spatial and temporal variations in crop status. However, satellites often have an inadequate spatial resolution for precision agriculture applications. High-resolution Unmanned Aerial Vehicles (UAV) imagery can be obtained at flexible dates, but operational costs may limit the collection frequency. Th
This paper studies the Front End of Eco-Innovation (FEEI), the initial phase of the eco-innovation process. Incorporating environmental concerns at the front-end of innovation is important, as product parameters are still flexible. This paper investigates the FEEI for 42 small and medium sized eco-innovators in the Netherlands by using a survey. The results show that SMEs embrace informal, systema
