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This paper presents the distributed architecture of the WIDE workjlow management system. We show how distribution and scalability are obtained by the use of a distributed object model, a clientlserver architecture, and a distributed workjlow server architecture. Specific attention is paid to the extended transaction support and active rule support(More)
This paper builds a conceptual framework to explain the obstacles that prevent Argentine producers of differentiated products from establishing a consistent presence in the developed world. We build our framework based on four case studies of sectoral export emergence in Argentina. We find that exporting consistently to developed countries requires drastic(More)
Spinal muscular atrophy is an autosomal-recessive neuromuscular disease caused by disruption of the survival of motor neuron (SMN) gene, which promotes cytoplasmic assembly of the splicing core machinery. It remains unclear how a deficiency in SMN results in a disorder leading to selective degeneration of lower motor neurons. We report here that SMN(More)
SMN1, the causative gene for spinal muscular atrophy (SMA), plays a housekeeping role in the biogenesis of small nuclear RNA ribonucleoproteins. SMN is also present in granular foci along axonal projections of motoneurons, which are the predominant cell type affected in the pathology. These so-called RNA granules mediate the transport of specific mRNAs(More)
This paper presents a workflow specification language developed in the WIDE project. The language provides a rich organisation model, an information model including presentation details, and a sophisticated process model. Workflow application developers should find the language a useful and compact means to capture and investigate design details. Workflow(More)
Spinal muscular atrophy (SMA), a lethal neurodegenerative disease that occurs in childhood, is caused by the misexpression of the survival of motor neuron (SMN) protein in motor neurons. It is still unclear whether activating motor units in SMA corrects the delay in the postnatal maturation of the motor unit resulting in an enhanced neuroprotection. In the(More)
1. Abstract Fingerprint recognition is one of the most popular methods used for identification with greater degree of success. The fingerprint has unique characteristics called minutiae, which are points where a curve track finishes, intersect or branches off. Identification systems using fingerprints biometric patterns are called AFIS (Automatic(More)
When cells are exposed to a genotoxic stress, a DNA surveillance pathway that involves p53 is activated, allowing DNA repair. Eukaryotic cells have also evolved a mechanism called mRNA surveillance that controls the quality of mRNAs. Indeed, mutant mRNAs carrying premature translation termination codons (PTCs) are selectively degraded by the(More)
UNLABELLED Spinal muscular atrophy (SMA) is a neuromuscular disease characterized by the selective loss of spinal motor neurons due to the depletion of the survival of motor neuron (SMN) protein. No therapy is currently available for SMA, which represents the leading genetic cause of death in childhood. In the present study, we report that insulin-like(More)