Semi-Supervised Learning of Cartesian Factors

The existence of place cells (PCs), grid cells (GCs), border cells (BCs), and head direction cells (HCs) as well as the dependencies between them have been enigmatic. We make an effort to explain their nature by introducing the concept of Cartesian Factors. These factors have specific properties: (i) they assume and complement each other, like direction and position and (ii) they have localized discrete representations with predictive attractors enabling implicit metric-like computations. In our model, HCs make the distributed and local representation of direction. Predictive attractor dynamics on that network forms the Cartesian Factor "direction." We embed these HCs and idiothetic visual information into a semi-supervised sparse autoencoding comparator structure that compresses its inputs and learns PCs, the distributed local and direction independent (allothetic) representation of the Cartesian Factor of global space. We use a supervised, information compressing predictive algorithm and form direction sensitive (oriented) GCs from the learned PCs by means of an attractor-like algorithm. Since the algorithm can continue the grid structure beyond the region of the PCs, i.e.,beyond its learning domain, thus the GCs and the PCs together form our metric-like Cartesian Factors of space. We also stipulate that the same algorithm can produce BCs. Our algorithm applies (a) a bag representation that models the "what system" and (b) magnitude ordered place cell activities that model either the integrate-and-fire mechanism, or theta phase precession, or both. We relate the components of the algorithm to the entorhinal-hippocampal complex and to its working. The algorithm requires both spatial and lifetime sparsification that may gain support from the two-stage memory formation of this complex

Xeroderma pigmentosum: overview of pharmacology and novel therapeutic strategies for neurological symptoms

Xeroderma Pigmentosum (XP) encompasses a group of rare diseases characterised in most cases by nucleotide excision repair (NER) malfunction, resulting in an increased sensitivity to ultraviolet radiation in affected individuals. Approximately 25-30% of XP patients present with neurological symptoms, such as sensorineural deafness, mental deterioration, and ataxia. Although it is known that dysfunctional DNA repair is the primary pathogenesis in XP, growing evidence suggests that mitochondrial pathophysiology may also occur. This appears to be secondary to dysfunctional NER but may contribute to the neurodegenerative process in these patients. The available pharmacological treatments in XP mostly target the dermal manifestations of the disease. In the present review, we outline how current understanding of the pathophysiology of XP could be used to develop novel therapies to counteract the neurological symptoms. Moreover, the coexistence of cancer and neurodegeneration present in XP, lead us to focus on possible new avenues targeting mitochondrial pathophysiology

Non-destructive structural analysis of the heat conducting path in power electronics and solid state lighting by thermal transient testing

Thermal transient testing is a tool for examining structural details of a heat conducting path composed of heat conduction and heat convection sections. Using the change of inherent heat in electronics, temperature transients provide a characterisation technique where using x-ray or acoustic microscopy would be troublesome and time-consuming. A complete toolkit for the accurate dynamic characterisation of subassemblies and cooling mounts is described in the paper. After giving the theoretical background, it is shown that the methodology can directly generate a valid and detailed structural equivalent from a single transient measurement. The descriptive thermal functions such as time constants and structure functions are demonstrated in practical examples, the capability of the structure functions for validating cooling concepts and detecting assembly problems is shown. Recognising the fact that systems with multiple energy transport such as electric energy converted to heat and light exhibit special features; the concept of structure functions has been modified for applications in solid state lighting.Papers presented at the 13th International Conference on Heat Transfer, Fluid Mechanics and Thermodynamics, Portoroz, Slovenia on 17-19 July 2017 .International centre for heat and mass transfer.American society of thermal and fluids engineers

Traces of Cu, Mn and Zn in Aquatic Animals, Water and Sediments from the Cris River Basin West Romania. Part II: Distribution Study

The paper presents the determination of Cu, Mn and Zn (TRT r.f.CCP-AES, FAAS) in water, sediment and aquatic animals (fish fillet, freshwater molluscs gills and muscles) collected in the basin of the Cris rivers (White Cris and Black Cris) in West Romania. The concentrations in water were in the range: 1-15 pg Lr1 (Cu), 10-1500 pg L-1 (Mn), 3-100 pg L-1 (Zn). Metal contents, as dry mass fractions, w^m x IO6, were: 5-380 (Cu), 225-2000 (Mn), 23-1140 (Zn) in sediment; 1-11 (Cu), 4-40 (Mn), 8-130 (Zn) in fish fillet, and 5-34 (Cu), 100-600 (Mn) and 50-130 (Zn) in mollusc muscles with higher values for the White Cris samples. Among the seven sample collection sites only one was identified as having concentrations of Mn and Zn in water higher than the admitted levels, but the limits of tolerance for aquatic organisms were not exceeded in either river. In mollusc gills, the metal contents expressed as wdm x IO6 were: 8-60 (Cu), 11000-16000 (Mn), 190-1200 (Zn), and similar for both rivers in the case of Mn

Erythropoietic protoporphyria

Erythropoietic protoporphyria (EPP) is an inherited disorder of the haem metabolic pathway characterised by accumulation of protoporphyrin in blood, erythrocytes and tissues, and cutaneous manifestations of photosensitivity. EPP has been reported worldwide, with prevalence between 1:75,000 and 1:200,000. It usually manifests in early infancy upon the first sun exposures. EPP is characterised by cutaneous manifestations of acute painful photosensitivity with erythema and oedema, sometimes with petechiae, together with stinging and burning sensations upon exposure to sunlight, without blisters. These episodes have a variable severity depending on the exposure duration and may result in chronic permanent lesions on exposed skin. As protoporphyrin is a lipophilic molecule that is excreted by the liver, EPP patients are at risk of cholelithiasis with obstructive episodes, and chronic liver disease that might evolve to rapid acute liver failure. In most patients, EPP results from a partial deficiency of the last enzyme of the haem biosynthetic pathway, ferrochelatase, EC 4.99.1.1/FECH (encoded by the FECH gene). EPP appears to be inherited as an autosomal dominant disease, the clinical expression of which is modulated by the presence of the hypomorphic FECH IVS3-48C allele trans, but recessive inheritance with two mutated FECH alleles has also been described. In about 2% of patients, overt disease was recently shown to be caused by gain-of-function mutations in the erythroid-specific aminolevulinic acid synthase 2 (ALAS2/ALAS, EC 2.3.1.27) gene and named X-linked dominant protoporphyria. Diagnosis is established by finding increased levels of protoporphyrin in plasma and red blood cells, and detection of a plasma fluorescence peak at 634 nm. Investigations for hepatic involvement, ferrochelatase activity level, genetic analysis (FECH mutations, presence of the hypomorphic FECH IVS3-48C allele trans and ALAS2 mutations) and family studies are advisable. Differential diagnosis includes phototoxic drug reactions, hydroa vacciniforme, solar urticaria, contact dermatitis, angio-oedema and, in some cases, other types of porphyria. Management includes avoidance of exposure to light, reduction of protoporphyrin levels and prevention of progression of possible liver disease to liver failure. As the major risk in EPP patients is liver disease, a regular follow-up of hepatic involvement is essential. Sequential hepatic and bone marrow transplantation should be considered as a suitable treatment for most severe cases of EPP with hepatic involvement. EPP is a lifelong disorder whose prognosis depends on the evolution of the hepatic disease. However, photosensitivity may have a significant impact on quality of life of EPP patients

An overview of the cutaneous porphyrias

This is an overview of the cutaneous porphyrias. It is a narrative review based on the published literature and my personal experience; it is not based on a formal systematic search of the literature. The cutaneous porphyrias are a diverse group of conditions due to inherited or acquired enzyme defects in the porphyrin–haem biosynthetic pathway. All the cutaneous porphyrias can have (either as a consequence of the porphyria or as part of the cause of the porphyria) involvement of other organs as well as the skin. The single commonest cutaneous porphyria in most parts of the world is acquired porphyria cutanea tarda, which is usually due to chronic liver disease and liver iron overload. The next most common cutaneous porphyria, erythropoietic protoporphyria, is an inherited disorder in which the accumulation of bile-excreted protoporphyrin can cause gallstones and, rarely, liver disease. Some of the porphyrias that cause blistering (usually bullae) and fragility (clinically and histologically identical to porphyria cutanea tarda) can also be associated with acute neurovisceral porphyria attacks, particularly variegate porphyria and hereditary coproporphyria. Management of porphyria cutanea tarda mainly consists of visible-light photoprotection measures while awaiting the effects of treating the underlying liver disease (if possible) and treatments to reduce serum iron and porphyrin levels. In erythropoietic protoporphyria, the underlying cause can be resolved only with a bone marrow transplant (which is rarely justifiable in this condition), so management consists particularly of visible-light photoprotection and, in some countries, narrowband ultraviolet B phototherapy. Afamelanotide is a promising and newly available treatment for erythropoietic protoporphyria and has been approved in Europe since 2014

Effect of Particle Size and Support Type on Pd Catalysts for 1,3-Butadiene Hydrogenation

Pd nanoparticles supported on SiO 2 , Si 3 N 4 and Al 2 O 3 were studied to examine the effect of particle size and support type on the hydrogenation of 1,3-butadiene. Pd nanoparticles were produced using a reverse micelle method resulting in particles with a remarkably small particle size distribution (σ < < 1 nm). The support type and particle size were observed to affect both catalytic activity and product selectivity. All catalysts showed a decrease of their activity with time on stream, paired with an increase in selectivity to butenes (1-butene and cis/trans-2-butene) from a product stream initially dominated by n-butane. In situ XAFS demonstrated a correlation between the formation of palladium hydride and n-butane production in the early stages (~ 1 h) of reaction. The extent of palladium hydride formation, as well as its depletion with time on stream, was dependent on both particle size and support type. Metallic Pd was identified as the species selective towards the production of butenes