Search Results - "Clarke, T R"

Natural course of Fabry disease: changing pattern of causes of death in FOS - Fabry Outcome Survey by Mehta, A, Clarke, J T R, Giugliani, R, Elliott, P, Linhart, A, Beck, M, Sunder-Plassmann, G

“…Fabry disease is a rare X-linked lysosomal storage disorder characterised by severe multisystemic involvement that leads to major organ failure and premature…”
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Successful Conservation of a Threatened Maculinea Butterfly by Thomas, J.A, Simcox, D.J, Clarke, R.T

“…Globally threatened butterflies have prompted research-based approaches to insect conservation. Here, we describe the reversal of the decline of Maculinea…”
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Comparative Losses of British Butterflies, Birds, and Plants and the Global Extinction Crisis by Thomas, J. A., Telfer, M. G., Roy, D. B., Preston, C. D., Greenwood, J. J. D., Asher, J., Fox, R., Clarke, R. T., Lawton, J. H.

“…There is growing concern about increased population, regional, and global extinctions of species. A key question is whether extinction rates for one group of…”
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Urinary Globotriaosylsphingosine-Related Biomarkers for Fabry Disease Targeted by Metabolomics by Auray-Blais, Christiane, Boutin, Michel, Gagnon, René, Dupont, Félix O, Lavoie, Pamela, Clarke, Joe T. R

“…Fabry disease is a lysosomal storage disorder caused by deficiency of α-galactosidase A, resulting in glycosphingolipid accumulation in organs and tissues,…”
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Pyrimethamine as a Potential Pharmacological Chaperone for Late-onset Forms of GM2 Gangliosidosis by Maegawa, Gustavo H.B., Tropak, Michael, Buttner, Justin, Stockley, Tracy, Kok, Fernando, Clarke, Joe T.R., Mahuran, Don J.

“…Late-onset GM2 gangliosidosis is composed of two related, autosomal recessive, neurodegenerative diseases, both resulting from deficiency of lysosomal,…”
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Application of a Policy Framework for the Public Funding of Drugs for Rare Diseases by Winquist, Eric, Coyle, Doug, Clarke, Joe T. R., Evans, Gerald A., Seager, Christine, Chan, Winnie, Martin, Janet

“…ABSTRACT BACKGROUND In many countries, decisions about the public funding of drugs are preferentially based on the results of randomized trials. For truly rare…”
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High-risk screening for Fabry disease in a Canadian cohort of chronic kidney disease patients by Auray-Blais, Christiane, Lavoie, Pamela, Abaoui, Mona, Côté, Anne-Marie, Boutin, Michel, Akbari, Ayub, Levin, Adeera, Mac-Way, Fabrice, TR Clarke, Joe

“…[Display omitted] •A high-risk screening study was performed for patients with chronic kidney disease.•Globotriaosylceramide (Gb3) was analyzed in dried urine…”
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The quality and isolation of habitat patches both determine where butterflies persist in fragmented landscapes by Thomas, J. A., Bourn, N. A. D., Clarke, R. T., Stewart, K. E., Simcox, D. J., Pearman, G. S., Curtis, R., Goodger, B.

“…and quantified the variation in carrying capacity within each butterfly's niche. We then made detailed surveys to compare the distribution and density of every…”
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Childhood onset of Scheie syndrome, the attenuated form of mucopolysaccharidosis I by Thomas, Janet A, Beck, Michael, Clarke, Joe T. R, Cox, Gerald F

“…Scheie syndrome is the most attenuated and rarest form of mucopolysaccharidosis type I (MPS I), an inherited lysosomal storage disorder. Only small patient…”
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Identification and Characterization of Ambroxol as an Enzyme Enhancement Agent for Gaucher Disease by Maegawa, Gustavo H.B., Tropak, Michael B., Buttner, Justin D., Rigat, Brigitte A., Fuller, Maria, Pandit, Deepangi, Tang, Liangiie, Kornhaber, Gregory J., Hamuro, Yoshitomo, Clarke, Joe T.R., Mahuran, Don J.

“…Gaucher disease (GD), the most prevalent lysosomal storage disease, is caused by a deficiency of glucocerebrosidase (GCase). The identification of small…”
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Spectral and thermal sensing for nitrogen and water status in rainfed and irrigated wheat environments by Fitzgerald, G.J, Rodriguez, D, Christensen, L.K, Belford, R, Sadras, V.O, Clarke, T.R

“…Variable-rate technologies and site-specific crop nutrient management require real-time spatial information about the potential for response to in-season crop…”
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Effects of locally rare taxa on the precision and sensitivity of RIVPACS bioassessment of freshwaters by CLARKE, R. T., MURPHY, J. F.

“…Summary 1. An overall aim in freshwater bioassessment is to use biological methods, metrics and forms of indices which are precise, in that they give…”
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The use of Bayesian methods for fitting rating curves, with case studies by Moyeed, R.A., Clarke, R.T.

“…Using data from two very large watersheds and five smaller, this paper explores the use of Bayesian methods for fitting rating curves. Posterior distribution…”
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Water use, crop coefficients, and irrigation management criteria for camelina production in arid regions by Hunsaker, D. J, French, A. N, Clarke, T. R, El-Shikha, D. M

“…Camelina sativa (L.) Crantz is an oilseed crop touted as being suitable for production in the arid southwestern USA. However, because any significant…”
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Narrative review : Fabry disease by CLARKE, Joe T. R

“…Fabry disease is an X-linked, hereditary, lysosomal storage disease caused by deficiency of the enzyme alpha-galactosidase A, which results in the accumulation…”
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Are groundwater nitrate concentrations reaching a turning point in some chalk aquifers? by Smith, J.T., Clarke, R.T., Bowes, M.J.

“…In past decades, there has been much scientific effort dedicated to the development of models for simulation and prediction of nitrate concentrations in…”
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Uncertainty in the estimation of mean annual flood due to rating-curve indefinition by Clarke, R.T

“…When rating-curves of the form Q= γ( h+ α) β are fitted by least-squares, goodness of fit as measured by the coefficient of determination r 2 is often close to…”
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Long Distance Seed Dispersal by Wind: Measuring and Modelling the Tail of the Curve by Bullock, James M., Clarke, Ralph T.

“…The size and shape of the tail of the seed dispersal curve is important in determining the spatial dynamics of plants, but is difficult to quantify. We devised…”
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Hydrological prediction in a non-stationary world by Clarke, R.T

“…The paper discusses evidence that common assumptions in the analysis of hydrological time series (homogeneous variability in random fluctuations about a…”
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Further evidence of changes in the hydrological regime of the River Paraguay: part of a wider phenomenon of climate change? by Collischonn, W, Tucci, C.E.M, Clarke, R.T

“…Analysis of flow measured at 20 sites, rainfall measured at 36 sites, and a 95-year record of water-level at one site in the basin of the River Paraguay (area…”
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