117,300 research outputs found
Hematopoietic stem cell transplantation for metachromatic leukodystrophy
No full textMetachromatic leukodystrophy (MLD) is a rare, fatal, inherited lysosomal storage disorder caused by the deficiency of arylsulfatase A (ARSA). The enzymatic defect results in the accumulation of the ARSA substrate, mainly in myelin forming cells, leading to progressive demyelination and dysfunction in the CNS and peripheral nervous system. The severity of the disease and the absence of approved treatments stimulate testing innovative therapeutic strategies.Areas covered: Among diverse experimental approaches, hematopoietic stem cell (HSC) transplantation (HSCT) from healthy compatible donors, and very recently, gene therapy based on transplantation of autologous HSCs transduced with a lentiviral vector encoding for the ARSA cDNA have been applied to MLD patients. State of the development of these HSC-based strategies is here analyzed, based on a review of the scientific literature, focusing on the outcomes of allogeneic HSCT, and on its potential clinical benefit and limitations.Expert opinion: HSC-based approaches would require the evaluation of long-term follow-up on neurological and transplant-related outcomes of larger cohorts of patients, in order to rationally define indication to treatment for MLD patients
Paralophaster lorioli
No full text<i>Paralophaster lorioli</i> (Koehler, 1907) <p>FIGURE 22 A–G</p> <p> <i>Solaster lorioli</i> Koehler, 1907: 558.</p> <p> <i>Paralophaster lorioli</i> Fisher 1940: 175; Madsen 1955: 14; H.E.S. Clark 1963: 60; Hurtado-Garcia & Manjón-Cabeza 2022: 1221.</p> <p> <b>Diagnosis</b></p> <p>Body strongly stellate, R/r=2.7–5.3 (Fig. 22A, C). Plates paxillate, 1 to 3 per plate, each with 4 to 12 slender, hyaline spinelets (Fig. 22A, D). Known specimens with 24–26 marginal paxillae per interradius (12–13 per arm) (Fig. 22C). Approximately 10–25 elongate, hyaline spines per inferomarginal paxillae at R= 1.6 cm. Furrow spines 2 to 3 webbing absent (Fig. 22F), subambulacral spines 4 to 5 with pronounced serrations, 7 to 12 along a single edge of each spine (Fig. 22E, G).</p> <p>Coelomic brooding of juveniles present (Fig. 22B).</p> <p> <b>Comments</b></p> <p> A widely occurring species which is present not only within the Southern Ocean but in the South Atlantic to abyssal depths (2897–4572 m). <i>Paralophaster lorioli</i> is distinguished from other <i>Paralophaster</i> by the pronounced serrations present on the subambulacral spines (Fig. 22F, G), which are absent from other known species. Function of these serrations is unknown.</p> <p> <i>Paralophaster lorioli</i> and <i>Paralophaster ferax</i> n. sp. display comparable characters in that the abactinal paxillae display weak overlap in spine number, 4 to 12 in the former versus 10−25 in the latter as well as similar numbers of marginal plates per arm side 12−13 versus 13−14 in <i>P. ferax</i> and nearly identical numbers of furrow spines, 2−3 versus 2−4. The two species differ most significantly in that known specimens of <i>P. ferax</i> lack the serrated subambulacral spines present on <i>Paralophaster lorioli</i> and have higher numbers of spinelets on their abactinal paxillae. Bathymetric occurrence of both species is overlapping (220−4572 m in <i>P. lorioli)</i> versus 3138–4429 m in <i>P. ferax</i>. Although the two species are conceivably synonyms, character differences are present and further evidence evaluating the two species is desirable.</p> <p> <b>Occurrence</b></p> <p> <b>Tierra Del Fuego</b>, <b>Drake Passage (Chile)</b>, Weddell Sea, <b>Elephant Island, Scotia Sea,</b> Scotia Arc, Antarctic Peninsula, Kerguelen, Ross Sea, Argentina, Bridgman Island, Ross Sea. <b>220</b> –4572 m.</p> <p> <b>Description</b></p> <p>Body strongly stellate (R/r=2.7–5.3). Disk sunken, interradial arcs acute. Arms petaloid, tapering distally (Fig. 22A, C).</p> <p>Abactinal surface composed of thickened lobate plates, many weakly articulated, forming widely reticulate network (Fig. 22B); some with trilobate to quadrilobate bases. Abactinal dermis prevalent. Plates paxillate, 1 to 3 per plate, each with 4 to 12 slender, hyaline spinelets. Single paxillae on individual plate. Paxillae irregularly, widely distributed over surface. Madreporite irregularly round, strongly convex, bounded by approximately 2 to 3 plates forming around the base.</p> <p>Marginal plates 18 (9 per arm side) at R=1.6 to 26 (13 per arm side) (Fig. 22C) per interradius at R= 2.5 to 3.9 cm. Only one marginal plate series is strongly defined and clearly observed, what is assumed to be the inferomarginal series owing to its position and near contact with the adambulacral plates. The presumptive superomarginals are weakly expressed with the plates while arranged serially, do not appear to be in close contact, especially those occurring distally, where they appear to be nearly absent. In contrast, the “inferomarginals” are very clearly observed and strongly expressed. These paxillae are widely spaced, thick, at least twice the thickness of the adjacent abactinal paxillae and cylindrical in cross-section. Approximately 10–25 elongate, hyaline spines per inferomarginal paxillae at R=1.6, 20–25 spines at R= 2.5 cm. Paxillar base of each inferomarginal similarly enlarged, thick with quadrate to round lobes. Inferomarginal plates sit in almost direct contact with adambulacral plate series.</p> <p>Actinal intermediate region filled almost entirely with membranous-tissue, no actinal plates observed (Fig. 22C).</p> <p>Adambulacral plates widely spaced, separated by membranous-tissue equal to the breadth of one or two adambulacral plates (Fig. 22F, G). Each adambulacral plate with 2 to 3 furrow spines (at R= 1.5 cm) to 4 to 5 furrow spines at R= 3.9 cm (Fig. 22F). Subambulacral spines 4 to 5, each with distinct, jagged serrations, approximately 6–12, along edge of each spine (Fig. 22E, G). Furrow spines on oral plates 10 with an 11th elongate spine directed into the mouth (thus a total of 20 furrow spines and 2 elongate spines directed into the mouth. Oral plates strongly arched, membranous tissue between first adambulacral and contact with oral plate.</p> <p> <b>Brooded juveniles</b></p> <p> CASIZ 167622 (Fig. 22B) from 361−363 m was discovered with approximately 8−12 brooding juveniles within the coelomic cavity with oral cavity facing upward toward the abactinal surface. This is the second <i>Paralophaster</i> species which demonstrates this behavior. Brooding in this species will be further detailed in a subsequent manuscript.</p> Material Examined <p> USNM 13474, Clarence Island, South Shetland Islands, Antarctica, Southern Ocean, −61.27, −54.75, 293–294 m. Coll. Squires & Pawson, R /V <i>Eastwind</i>, 17 Feb. 1966. 1 dry spec. R=3.3 r=0.5.</p> <p> USNM 1468788, Victoria Land, Southern Ocean, −70.542, 171.142, 2421 m. Coll. R/V <i>Eltanin</i>, USAP, 13 Feb. 1968. 1 dry specs. R=3.9 r=0.9.</p> <p> USNM 1468789, Drake Passage, Tierra del Fuego, South Atlantic Ocean, −57.217, −62.8, 3733–3806 m. Coll. R/V <i>Eltanin</i>, USAP, 29 July 1962. 1 dry spec. R=3.4 r=0.7.</p> <p> USNM 1660592, Scotia Sea, South Atlantic Ocean, −58.925, −53.983, 3817–3931 m. Coll. R/V <i>Eltanin</i>, USAP, 25 Jan. 1966. 3 dry specs. R=2.4 r=1.0, R=2.5 r=0.5, R=1.7 r=0.6.</p> <p> USNM 1660593, Southern Ocean, Antarctica. −73.317, −174.875, 2897–2907 m. Coll. R/V <i>Eltanin</i>, USAP, 9 Feb. 1968. 2 dry specs. R=2.9 r=1.0, R=2.5 r=1.9 (arm tips broken on latter specimen).</p> <p> USNM 1570997, Elephant Island, South Shetland Islands, Southern Ocean, Antarctica, −61.317, −56.158, 220– 240 m. Coll. R/V <i>Eltanin</i>, 31 Dec. 1962. 4 dry specs. R=2.6 r=0.9, R=1.7 r=0.4, R=1.8 r=0.4, R=2.9 r=0.6.</p> <p> USNM 1573491, Drake Passage, Chile, −56.942, −74.808, 4209 m. Coll. R/V <i>Eltanin</i> USAP, 11 Nov. 1963. 1 dry spec. R=1.9 r=0.7.</p> <p> CASIZ 167622, Off King George Island, South Shetland Island, − 61.775833, −57.390556, 361−363 m. Coll. R. Mooi & S. Lockhart, aboard R/V <i>Yuzhmorgeologiya</i>, 19 Aug. 2003. 1 wet spec. R=2.8 r=1.1.</p>Published as part of <i>Mah, Christopher L., 2023, New Genera, Species, and observations on the biology of Antarctic Valvatida (Asteroidea), pp. 1-88 in Zootaxa 5310 (1)</i> on pages 67-69, DOI: 10.11646/zootaxa.5310.1.1, <a href="http://zenodo.org/record/8090240">http://zenodo.org/record/8090240</a>
Going Beyond Counting First Authors in Author Co-citation Analysis
No full textThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Square Dancing with the Stars to Enhance Dynamic Hirschman Linkages?
No full textIn this Presidential Address, the author takes the reader on a reconnaissance of his life and time as a regional scientist. He points out scenery he found scintillating along the way, hoping that some may pick up the banner and chew on a few of the ideas for a while. He suggests a revisit to Albert O. Hirschman’s notion of key sectors and more empirical analysis related to Marcus Berliant’s and Masahisa Fujita’s notion of knowledge creation and transfer.Presidential Address, San Antonio, Texas, March 29, 2014 (53rd Meetings of the Southern Regional Science Association
Appropriate Similarity Measures for Author Cocitation Analysis
No full textWe provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
Letter from unknown writer to Jesse L. Boyce
No full textLetter to Jesse L. Boyce from unknown author (possibly Jack) about the investigation into the powder magazine located in the Grand Canyon. Some personal news is included in the letter such as the writer's marriage to the daughter of C.A. Taylor, former Supervisor of Cochise County
Dispelling the Myths Behind First-author Citation Counts
No full textWe conducted a full-scale evaluative citation analysis study of scholars in the XML research field to explore just how different from each other author rankings resulting from different citation counting methods actually are, and to demonstrate the capability of emerging data and tools on the Web in supporting more realistic citation counting methods. Our results contest some common arguments for the continued
use of first-author citation counts in the evaluation of scholars, such as high correlations between author rankings by first-author citation counts and other citation
counting methods, and high costs of using more realistic citation counting methods that are not well-supported by the ISI databases. It is argued that increasingly available digital full text research papers make it possible for citation analysis studies to go beyond what the ISI databases have directly supported and to employ more
sophisticated methods
Sarah L. Blum Author Visit - Warrior Nurse: PTSD and Healing
No full textHear Sarah L. Blum, author of Women Under Fire: Abuse in the Military, discuss her newest book, Warrior Nurse: PTSD and Healing followed by a Q&A and book signing.
Sarah L. Blum is a decorated Vietnam veteran who served as an operating room nurse during the intense fighting of 1967. In recognition of her service, she was awarded the Army Commendation Medal.
Sponsored by CWU Veterans Center and CWU Libraries.https://digitalcommons.cwu.edu/libraryevents/1252/thumbnail.jp
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