Salt Effects in the Solvolysis of Some Alkyl Chlorides in Different Solvent Systems

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A 15-year perspective of the fabry outcome survey

The Fabry Outcome Survey (FOS) is an international long-term observational registry sponsored by Shire for patients diagnosed with Fabry disease who are receiving or are candidates for therapy with agalsidase alfa (agala). Established in 2001, FOS provides long-term data on agala safety/efficacy and collects data on the natural history of Fabry disease, with the aim of improving clinical management. The FOS publications have helped establish prognostic and severity scores, defined the incidence of specific disease variants and implications for clinical management, described clinical manifestations in special populations, confirmed the high prevalence of cardiac morbidity, and demonstrated correlations between ocular changes and Fabry disease severity. These FOS data represent a rich resource with utility not only for description of natural history/therapeutic effects but also for exploratory hypothesis testing and generation of tools for diagnosis/management, with the potential to improve future patient outcomes

Novel peptides of therapeutic promise from Indian conidae

Highly structured small peptides are the major toxic constituents of the venom of cone snails, a family of widely distributed predatory marine molluscs. These animals use the venom for rapid prey immobilization. The peptide components in the venom target a wide variety of membrane-bound ion channels and receptors. Many have been found to be highly selective for a diverse range of mammalian ion channels and receptors associated with pain-signaling pathways. Their small size, structural stability, and target specificity make them attractive pharmacologic agents. A select number of laboratories mainly from the United States, Europe, Australia, Israel, and China have been engaged in intense drug discovery programs based on peptides from a few snail species. Coastal India has an estimated 20-30% of the known cone species; however, few serious studies have been reported so far. We have begun a comprehensive program for the identification and characterization of peptides from cone snails found in Indian Coastal waters. This presentation reviews our progress over the last 2 years. As expected from the evolutionary history of these venom components, our search has yielded novel peptides of therapeutic promise from the new species that we have studied

Identification and Structural Characterization of Interneurons of the Drosophila Brain by Monoclonal Antibodies of the Würzburg Hybridoma Library

Several novel synaptic proteins have been identified by monoclonal antibodies (mAbs) of the Würzburg hybridoma library generated against homogenized Drosophila brains, e.g. cysteine string protein, synapse-associated protein of 47 kDa, and Bruchpilot. However, at present no routine technique exists to identify the antigens of mAbs of our library that label only a small number of cells in the brain. Yet these antibodies can be used to reproducibly label and thereby identify these cells by immunohistochemical staining. Here we describe the staining patterns in the Drosophila brain for ten mAbs of the Würzburg hybridoma library. Besides revealing the neuroanatomical structure and distribution of ten different sets of cells we compare the staining patterns with those of antibodies against known antigens and GFP expression patterns driven by selected Gal4 lines employing regulatory sequences of neuronal genes. We present examples where our antibodies apparently stain the same cells in different Gal4 lines suggesting that the corresponding regulatory sequences can be exploited by the split-Gal4 technique for transgene expression exclusively in these cells. The detection of Gal4 expression in cells labeled by mAbs may also help in the identification of the antigens recognized by the antibodies which then in addition to their value for neuroanatomy will represent important tools for the characterization of the antigens. Implications and future strategies for the identification of the antigens are discussed

Presenting signs and patient co-variables in Gaucher disease : outcome of the Gaucher Earlier Diagnosis Consensus (GED-C) Delphi initiative

© 2018 The Authors. Internal Medicine Journal by Wiley Publishing Asia Pty Ltd on behalf of Royal Australasian College of Physicians.Background: Gaucher disease (GD) presents with a range of signs and symptoms. Physicians can fail to recognise the early stages of GD owing to a lack of disease awareness, which can lead to significant diagnostic delays and sometimes irreversible but avoidable morbidities. Aim: The Gaucher Earlier Diagnosis Consensus (GED-C) initiative aimed to identify signs and co-variables considered most indicative of early type 1 and type 3 GD, to help non-specialists identify ‘at-risk’ patients who may benefit from diagnostic testing. Methods: An anonymous, three-round Delphi consensus process was deployed among a global panel of 22 specialists in GD (median experience 17.5 years, collectively managing almost 3000 patients). The rounds entailed data gathering, then importance ranking and establishment of consensus, using 5-point Likert scales and scoring thresholds defined a priori. Results: For type 1 disease, seven major signs (splenomegaly, thrombocytopenia, bone-related manifestations, anaemia, hyperferritinaemia, hepatomegaly and gammopathy) and two major co-variables (family history of GD and Ashkenazi-Jewish ancestry) were identified. For type 3 disease, nine major signs (splenomegaly, oculomotor disturbances, thrombocytopenia, epilepsy, anaemia, hepatomegaly, bone pain, motor disturbances and kyphosis) and one major co-variable (family history of GD) were identified. Lack of disease awareness, overlooking mild early signs and failure to consider GD as a diagnostic differential were considered major barriers to early diagnosis. Conclusion: The signs and co-variables identified in the GED-C initiative as potentially indicative of early GD will help to guide non-specialists and raise their index of suspicion in identifying patients potentially suitable for diagnostic testing for GD.Peer reviewedFinal Published versio

Saturation in cascaded optical amplifier free-space optical communication systems

The performance of a free-space optical (FSO) communication system in a turbulent atmosphere employing an optical amplifier (OA) cascade to extend reach is investigated. Analysis of both single and cascaded OA FSO communication links is given and the implications of using both adaptive (to channel state) and non-adaptive decision threshold schemes are analysed. The benefits of amplifier saturation, for example in the form of effective scintillation reduction when a non-adaptive decision threshold scheme is utilised at the receiver for different atmospheric turbulence regimes, are presented. Monte Carlo simulation techniques are used to model the probability distributions of the optical signal power, noise and the average bit error rate due to scintillation for the cascade. The performance of an adaptive decision threshold is superior to a non-adaptive decision threshold for both saturated and fixed gain preamplified receivers and the ability of a saturated gain OA to suppress scintillation is only meaningful for system performance when a non-adaptive decision threshold is used at the receiver. An OA cascade can be successfully used to extend reach in FSO communication systems and specific system implementations are presented. The optimal cascade scheme with a non-adaptive receiver would use frequent low gain saturated amplification

Schemes of transmission of classical information via quantum channels with many senders: discrete and continuous variables cases

Superadditivity effects in the classical capacity of discrete multi-access channels (MACs) and continuous variable (CV) Gaussian MACs are analysed. New examples of the manifestation of superadditivity in the discrete case are provided including, in particular, a channel which is fully symmetric with respect to all senders. Furthermore, we consider a class of channels for which {\it input entanglement across more than two copies of the channels is necessary} to saturate the asymptotic rate of transmission from one of the senders to the receiver. The 5-input entanglement of Shor error correction codewords surpass the capacity attainable by using arbitrary two-input entanglement for these channels. In the CV case, we consider the properties of the two channels (a beam-splitter channel and a "non-demolition" XP gate channel) analyzed in [Czekaj {\it et al.}, Phys. Rev. A {\bf 82}, 020302 (R) (2010)] in greater detail and also consider the sensitivity of capacity superadditivity effects to thermal noise. We observe that the estimates of amount of two-mode squeezing required to achieve capacity superadditivity are more optimistic than previously reported.Comment: 19 pages, 11 figure