1,800 research outputs found
Anatomical changes and pathophysiology of the brain in mucopolysaccharidosis disorders
Full text linkMucopolysaccharidosis (MPS) disorders are caused by deficiencies in lysosomal enzymes, leading to impaired glycosaminoglycan (GAG) degradation. The resulting GAG accumulation in cells and connective tissues ultimately results in widespread tissue and organ dysfunction. The seven MPS types currently described are heterogeneous and progressive disorders, with somatic and neurological manifestations depending on the type of accumulating GAG. Heparan sulfate (HS) is one of the GAGs stored in patients with MPS I, II, and VII and the main GAG stored in patients with MPS III. These disorders are associated with significant central nervous system (CNS) abnormalities that can manifest as impaired cognition, hyperactive and/or aggressive behavior, epilepsy, hydrocephalus, and sleeping problems. This review discusses the anatomical and pathophysiological CNS changes accompanying HS accumulation as well as the mechanisms believed to cause CNS abnormalities in MPS patients. The content of this review is based on presentations and discussions on these topics during a meeting on the brain in MPS attended by an international group of MPS experts
Oral Migalastat HCl Leads to Greater Systemic Exposure and Tissue Levels of Active α-Galactosidase A in Fabry Patients when Co-Administered with Infused Agalsidase.
Full text linkMigalastat HCl (AT1001, 1-Deoxygalactonojirimycin) is an investigational pharmacological chaperone for the treatment of α-galactosidase A (α-Gal A) deficiency, which leads to Fabry disease, an X-linked, lysosomal storage disorder. The currently approved, biologics-based therapy for Fabry disease is enzyme replacement therapy (ERT) with either agalsidase alfa (Replagal) or agalsidase beta (Fabrazyme). Based on preclinical data, migalastat HCl in combination with agalsidase is expected to result in the pharmacokinetic (PK) enhancement of agalsidase in plasma by increasing the systemic exposure of active agalsidase, thereby leading to increased cellular levels in disease-relevant tissues. This Phase 2a study design consisted of an open-label, fixed-treatment sequence that evaluated the effects of single oral doses of 150 mg or 450 mg migalastat HCl on the PK and tissue levels of intravenously infused agalsidase (0.2, 0.5, or 1.0 mg/kg) in male Fabry patients. As expected, intravenous administration of agalsidase alone resulted in increased α-Gal A activity in plasma, skin, and peripheral blood mononuclear cells (PBMCs) compared to baseline. Following co-administration of migalastat HCl and agalsidase, α-Gal A activity in plasma was further significantly increased 1.2- to 5.1-fold compared to agalsidase administration alone, in 22 of 23 patients (95.6%). Importantly, similar increases in skin and PBMC α-Gal A activity were seen following co-administration of migalastat HCl and agalsidase. The effects were not related to the administered migalastat HCl dose, as the 150 mg dose of migalastat HCl increased α-Gal A activity to the same extent as the 450 mg dose. Conversely, agalsidase had no effect on the plasma PK of migalastat. No migalastat HCl-related adverse events or drug-related tolerability issues were identified.ClinicalTrials.gov NCT01196871
Protein structure and function / Brian W. Matthews
Full text linkConsists of offprints of the author's articlesIncludes bibliographies1 v. (various pagings) :This electronic version is made publicly available by the University of Adelaide in accordance with its open access policy for student theses. Copyright in this thesis remains with the author. This thesis may incorporate third party material which has been used by the author pursuant to Fair Dealing exceptions. If you are the owner of any included third party copyright material you wish to be removed from this electronic version, please complete the take down form located at: http://www.adelaide.edu.au/legalsThesis (D. Sc.)--University of Adelaide, 198
Preliminary data on the influence of rearing temperature on the growth and reproductive status of fathead minnows Pimephales promelas
Full text linkThis article has been made available through the Brunel Open Access Publishing Fund and is available from the specified link - Copyright @ 2011 Brian JV et al.An investigation into the influence of temperature on the growth and reproductive status of the fathead minnow Pimephales promelas revealed that, while there was no clear effect of treatment on sex differentiation, ovarian tissue from female fish reared under the highest temperature regime contained large amounts of undefined tissue containing no germ cells. Furthermore, both male and female fish exhibited differences in length mass, condition and somatic indices, and in the expression of secondary sexual characteristics. The patterns observed are discussed in the context of climate change
A compendium of Grande Ronde River and Imnaha River basins spring chinook salmon spawning ground surveys conducted from 1948 through 2003
No full textJ. Vincent Tranquilli, Brian C. Jonasson, MaryLouise, Keefe Richard W. Carmichael.Title from PDF title page (viewed on February 16, 2023).This archived document is maintained by the State Library of Oregon as part of the Oregon Documents Depository Program. It is for informational purposes and may not be suitable for legal purposes.Includes bibliographical references (pages 33-40).Financed by the U.S. Fish and Wildlife Service under the Lower Snake River Compensation Plan.Mode of access: Internet from the Oregon Government Publications Collection.Text in English
The application of system engineering methodologies in support of the lean enterprise transformation
No full textThesis (S.M.)--Massachusetts Institute of Technology, System Design & Management Program, 2001.This electronic version was submitted by the student author. The certified thesis is available in the Institute Archives and Special Collections.Includes bibliographical references (leaves 110-111).by David L. Cocuzzo and Brian W. Millard.S.M
Applicability of evolutionary algorithms for orbit optimization in the strongly perturbed environment of the 2001 SN263 triple asteroid system
No full textThe ASTER mission under study by the Brazilian National Institute for Space Research would be the first to explore a triple asteroid system. To find orbits stable in such a perturbed environment, an orbit propagation and optimization programme has been written, which makes use of Evolutionary evolution algorithms. The programme written proves sufficient to identify suitable solutions for several use cases based on various mission phases and scenarios. The solar radiation pressure has been identified as a critical perturbation that prevents the existence of solutions in some scenarios, and often drives existing solutions towards terminator orbits.Green Open Access added to TU Delft Institutional Repository ‘You share, we take care!’ – Taverne project https://www.openaccess.nl/en/you-share-we-take-care Otherwise as indicated in the copyright section: the publisher is the copyright holder of this work and the author uses the Dutch legislation to make this work public.Astrodynamics & Space Mission
Emperor and author : the writings of Julian the Apostate /
No full textIncludes bibliographical references and index.Introduction Nicholas Baker-Brian and Shaun Tougher -- Julian the writer and his audience / Susanna Elm -- Reading between the lines : ; Julian's First Panegyric on Constantius II / Shaun Tougher -- 'But I digress...' : ; rhetoric and propaganda in Julian's second oration to Constantius / Hal Drake -- Is there an empress in the text? ; Julian's Speech of thanks to Eusebia / Liz James -- Julian's Consolation to himself on the departure of the excellent Salutius : ; rhetoric and philosophy in the fourth centurry / Josef Lössl -- The tyrant's mask? ; Images of good and bad rule in Julian's Letter to the Athenians / Mark Humphries -- Julian's Letter to Themistius -- and Themistius' response? / John W. Watt -- The emperor's shadow : ; Julian in his correspondence / Michael Trapp -- Julian the lawgiver / Jill Harries -- Words and deeds : ; Julian in the epigraphic record / Benet Salway -- Julian and his coinage : ; a very Constantinian prince / Fernando López Sánchez -- Roman authority, imperial authoriality, and Julian's artistic program / Eric R. Varner -- Julian's Hymn to the mother of the gods : ; the revival and justification of traditional religion / J.H.W.G. Liebeschuetz -- Julian's Hymn to King Helios : ; the economical use of complex Neoplatonic concepts / Andrew Smtih -- The forging of an Hellenic orthodoxy : ; Julian's speeches against the cynics / Arnaldo Marcone -- The Christian context of Julian's Against the Galileans / David Hunt -- The politics of virtue in Julian's Misopogon / Nicholas Baker-Brian -- The Caesars of Julian the Apostate in translation and reception, 1580-ca -- 1800 / Rowland SmithAfterword: studying Julian the author / Jacqueline Long
Supernova shrapnel: nearby supernovae and dust transport in the ISM
Full text linkThe last 2 decades have seen the proposal, detection, and confirmation of live ^60Fe radioisotopes from an extra-solar source on Earth, showing an event outside the Solar System directly delivered material to the Earth since its formation. This work examines the possible sources for the ^60Fe and models the passage of the material from its source through the Solar System to the ocean floor. We consider the production and deposition on Earth of isotopes with half-lives in the range 10^5 to 10^8 years that might provide signatures of nearby stellar explosions, extending previous analyses of Core-Collapse Supernovae (CCSNe) to include Electron-Capture Supernovae (ECSNe), Super-Asymptotic Giant Branch (SAGBs) stars, Thermonuclear/Type Ia Supernovae (TNSNe), and Kilonovae/Neutron Star Mergers (KNe). We revisit previous estimates of the ^60Fe and ^26Al signatures, and extend these estimates to include ^244Pu and ^53Mn. We show that (i) the ^60Fe yield rules out the TNSN and KN interpretations, (ii) the ^60Fe signals highly constrain a SAGB interpretation but do not completely them rule out, (iii) are consistent with a CCSN origin, and (iv) are highly compatible with an ECSN interpretation.
We also examine various influences on the path of interstellar dust carrying ^60Fe from a SN through the Heliosphere, with the aim of estimating the final global distribution on the ocean floor. We study the influences of magnetic fields, angle of arrival, wind and ocean cycling of SN material on the concentrations at different locations. We find that the passage of SN material through the mesosphere/lower thermosphere (MLT) is the greatest influence on the final global distribution, with ocean cycling causing lesser alteration as the SN material sinks to the ocean floor. SN distance estimates in previous works that assumed a uniform distribution are a good approximation. Including the effects on surface distributions, we estimate a distance of 46^(+10)_(-6) pc for an ECSN progenitor. We note that the SN dust retains directional information to within 1^(circ) through its arrival in the inner Solar System, so that SN debris deposition on inert bodies such as the Moon will be anisotropic, and thus could in principle be used to infer directional information.
Lastly, we examine the various influences on the path of dust within a SN remnant (SNR) to determine when/if the dust decouples from the plasma, how much it is sputtered, and where within the ejecta the dust is located. We find that the inclusion of Rayleigh-Taylor (R-T) instabilities are important in studying dust survival as R-T instabilities influence the location of the SN's reverse shock. We also find the presence of a magnetic field within the shocked ISM material will limit the passage of SN dust grains reflecting them or trapping within the heart of the SNR.Submission published under a 24 month embargo labeled 'U of I Access', the embargo will last until 2018-08-01The student, Brian Fry, accepted the attached license on 2016-07-04 at 15:08.The student, Brian Fry, submitted this Dissertation for approval on 2016-07-04 at 15:23.This Dissertation was approved for publication on 2016-07-08 at 16:47.DSpace SAF Submission Ingestion Package generated from Vireo submission #9750 on 2016-11-10 at 12:24:50Made available in DSpace on 2016-11-10T18:39:16Z (GMT). No. of bitstreams: 2
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Psychic Wholeness and Healing by Anna A. Terruwe and Conrad W. Baars
No full textThis paper is a review of the book: Anna A. Terruwe and Conrad W. Baars, Psychic Wholeness and Healing (Eugene, Oreg.: Wipf and Stock, 2016, 2nd edition). According to the author, the book shows (1) how Thomistic thought can be applied to both psychoanalytic theories and practical psychological and spiritual issues, and (2) what role is possible for religion to play in reinvigorating psychoanalysis
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