162,597 research outputs found
[Report to Chief J. E. Curry, by an unknown author #1]
No full textReport to Chief J. E. Curry, by an unknown author. The report contains a list of officers who gave depositions to the United States Attorney
[Report to Chief J. E. Curry, by an unknown author #2]
No full textReport to Chief J. E. Curry, by an unknown author. The report contains a list of officers who gave depositions to the United States Attorney
Studies on hereditary spastic paraplegia proteins
No full textThe hereditary spastic paraplegias (HSPs) are a clinically and genetically diverse group of inherited neurological disorders that primarily cause progressive spasticity and weakness in the lower limbs due to a length-dependent, retrograde degradation of the corticospinal motor neurons. Purely spastic paraplegia is also known as uncomplicated HSP, but complicated forms of HSP exist as well, with symptoms such as mental retardation, dementia, seizures, and optic, cortical, and cerebellar atrophy. Twenty gene products have been identified from over 40 different known SPG spastic gait loci (SPG1-46), which may be inherited in autosomal dominant, autosomal recessive, or X-linked manners.The work presented in this thesis focuses on two different HSP proteins: atlastin-1, a member of the dynamin superfamily of large GTPases through sequence similarity, and maspardin Mast syndrome, spastic paraplegia, autosomal recessive with dementia. Mutations in the atlastin-1 gene, SPG3A, are the second most common cause of autosomal dominant HSP around 10% of all cases many of which are quite early in onset (in childhood) when compared to other forms of HSP. Diseasecausing point mutations are distributed throughout the coding region, although most are clustered in known domains GTP binding/GTPase functional areas, a coiled coil region in the middle of the protein product, and in the transmembrane areas at the Cterminal end. On the other hand, the only causative mutation in SPG21/MAST (maspardin) that has been found is a frameshift-producing alteration after the second third of the gene, which induces a premature truncation of the gene product and loss of the last 95 amino acids of the wild type protein. This mutation is only inherited in an autosomal recessive manner, and causes a complicated HSP with additional symptoms such as dementia, white matter abnormalities, and cerebellar and extrapyramidal signs.Atlastin-1 is localized to the ER and cis-Golgi apparatus in the adult brain, and appears to exist natively as oligomers, most likely tetramers. Wild-type atlastin-1 is a functional GTPase, but in paper I we found that several missense atlastin-1 mutations have impaired GTPase activity. We also found that atlastin-1 is highly enriched in vesicular structures within growth cones, varicosities, and axonal branch points. Knockdown of atlastin-1 using small hairpin RNAs impairs axon formation and elongation during neuronal development and reduces the total number of neuronal processes. In paper II we examined a novel SPG3A mutation causative for HSP that did not affect GTPase activity or interactions between atlastin and spastin, the gene most mutated in HSP. However, immunoblots from patient lymphoblasts showed a reduction in atlastin-1 protein levels, indicating that mutant atlastin-1 may cause disease pathogenesis through a dominant-negative, loss-of-function manner through protein destabilization.Mast syndrome is likely caused by a loss of protein function. In paper IV we generated SPG21-/- transgenic mice as a possible model for SPG21. Though SPG21-/- mice appeared normal at birth, within several months they developed a mild but progressive hind limb dysfunction. Cultured cerebral cortical neurons from SPG21-/- mice exhibited significantly more axonal branching than neurons cultured from wildtype animals, although a comprehensive neuropathological analysis did not reveal any abnormalities consistent with those observed in human HSP. While a unifying mechanism for all the genes and proteins known to be involved in HSP has yet to be found, our data support the idea that axonal trafficking and proper neurite branching may represent a common cellular pathogenic theme.List of scientific papersI. Zhu PP, Soderblom C, Tao-Cheng JH, Stadler J, Blackstone C (2006). "SPG3A protein atlastin-1 is enriched in growth cones and promotes axon elongation during neuronal development." Hum Mol Genet 15(8): 1343-53. Epub 2006 Mar 14 https://pubmed.ncbi.nlm.nih.gov/16537571II. Meijer IA, Dion P, Laurent S, Dupré N, Brais B, Levert A, Puymirat J, Rioux MF, Sylvain M, Zhu PP, Soderblom C, Stadler J, Blackstone C, Rouleau GA (2007). "Characterization of a novel SPG3A deletion in a French-Canadian family." Ann Neurol 61(6): 599-603 https://pubmed.ncbi.nlm.nih.gov/17427918III. Rismanchi N, Soderblom C, Stadler J, Zhu PP, Blackstone C (2008). "Atlastin GTPases are required for Golgi apparatus and ER morphogenesis." Hum Mol Genet 17(11): 1591-604. Epub 2008 Feb 12 https://pubmed.ncbi.nlm.nih.gov/18270207IV. Soderblom C, Stadler J, Jupille H, Blackstone C, Shupliakov O, Hanna MC (2010). "Targeted disruption of the Mast syndrome gene SPG21 in mice impairs hind limb function and alters axon branching in cultured cortical neurons." (Submitted)</p
Murder on the mountain: author talk with Peter J. Wosh
No full textAuthor talk by Peter J. Wosh on May 5th, 2022, on his book, "Murder on the Mountain: crime, passion, and punishment in gilded age New Jersey.
Geology of the Selk crater region on Titan from Cassini VIMS observations
No full textInternational audienceObservations of Titan obtained by the Cassini Visual and Infrared Mapping Spectrometer (VIMS) have revealed Selk crater, a geologically young, bright-rimmed, impact crater located similar to 800 km north-northwest of the Huygens landing site. The crater rim-crest diameter is 90 km; its floor diameter is similar to 60 km. A central pit/peak, 20-30 km in diameter, is seen; the ratio of the size of this feature to the crater diameter is consistent with similarly sized craters on Ganymede and Callisto, all of which are dome craters. The VIMS data, unfortunately, are not of sufficient resolution to detect such a dome. The inner rim of Selk crater is fluted, probably by eolian erosion, while the outer flank and presumed ejecta blanket appear dissected by drainages (particularly to the east), likely the result of fluvial erosion. Terracing is observed on the northern and western walls of Selk crater within a 10-15 km wide terrace zone identified in VIMS data; the terrace zone is bright in SAR data, consistent with it being a rough surface. The terrace zone is slightly wider than those observed on Ganymede and Callisto and may reflect differences in thermal structure and/or composition of the lithosphere. The polygonal appearance of the crater likely results from two preexisting planes of weakness (oriented at azimuths of 21 degrees and 122 degrees east of north). A unit of generally bright terrain that exhibits similar infrared-color variation and contrast to Selk crater extends east-southeast from the crater several hundred kilometers. We informally refer to this terrain as the Selk "bench." Both Selk and the bench are surrounded by the infrared-dark Belet dune field. Hypotheses for the genesis of the optically bright terrain of the bench include: wind shadowing in the lee of Selk crater preventing the encroachment of dunes, impact-induced cryovolcanism, flow of a fluidized-ejecta blanket (similar to the bright crater outflows observed on Venus), and erosion of a streamlined upland formed in the lee of Selk crater by fluid flow. Vestigial circular outlines in this feature just east of Selk's ejecta blanket suggest that this might be a remnant of an ancient, cratered crust. Evidently the southern margin of the feature has sufficient relief to prevent the encroachment of dunes from the Belet dune field. We conclude that this feature either represents a relatively high-viscosity, fluidizedejecta flow (a class intermediate to ejecta blankets and long venusian-style ejecta flows) or a streamlined upland remnant that formed downstream from the crater by erosive fluid flow from the west-northwest
Mr. Melvin J. Collier, RWWL AUC, June 2011
No full textThis video is a conversation with Mr. Melvin J. Collier. Mr. Collier talks about his book, "From Mississippi to Africa: A Journey of Discovery". Daniel Le, AUC Woodruff Library, is the interviewer
A Tripartite Post-Recession Rebalancing
No full textIn this latest Advance & Rutgers Report, entitled “A Tripartite Post-Recession Rebalancing,” Dean James W. Hughes and Professor Joseph J. Seneca deliver an incisive assessment of the current market conditions and obstacles in the path of our economic recovery. They offer a statistical cautionary tale that the private and public sector need to hear and acknowledge in order for the economy to make continued progress.This report was published as Issue Paper Number 7, November 2011, in Advance & Rutgers Report
Evidence for the decay B0→J/ψω and measurement of the relative branching fractions of meson decays to J/ψη and J/ψη′
No full textFirst evidence of the B 0 → J / ψ ω decay is found and the B s 0 → J / ψ η and B s 0 → J / ψ η ′ decays are studied using a dataset corresponding to an integrated luminosity of 1.0 fb -1 collected by the LHCb experiment in proton-proton collisions at a centre-of-mass energy of sqrt(s) = 7 TeV. The branching fractions of these decays are measured relative to that of the B 0 → J / ψ ρ 0 decay:frac(B (B 0 → J / ψ ω), B (B 0 → J / ψ ρ 0)) = 0.89 ± 0.19 (stat) - 0.13 + 0.07 (syst),frac(B (B s 0 → J / ψ η), B (B 0 → J / ψ ρ 0)) = 14.0 ± 1.2 (stat) - 1.5 + 1.1 (syst) - 1.0 + 1.1 (frac(f d, f s)),frac(B (B s 0 → J / ψ η ′), B (B 0 → J / ψ ρ 0)) = 12.7 ± 1.1 (stat) - 1.3 + 0.5 (syst) - 0.9 + 1.0 (frac(f d, f s)), where the last uncertainty is due to the knowledge of f d / f s, the ratio of b-quark hadronization factors that accounts for the different production rate of B 0 and B s 0 mesons. The ratio of the branching fractions of B s 0 → J / ψ η ′ and B s 0 → J / ψ η decays is measured to befrac(B (B s 0 → J / ψ η ′), B (B s 0 → J / ψ η)) = 0.90 ± 0.09 (stat) - 0.02 + 0.06 (syst)
The vanishing author in computer-generated works: a critical analysis of recent Australian case law
No full textAbstract
The use of software is ubiquitous in the creation of many copyright works, yet the requirement in copyright law that every work have a human author who engages in independent intellectual effort means that its use may prevent copyright subsistence. Several recent Australian cases have refocused attention on authorship as an essential criterion of copyright subsistence, and these cases suggest that much computer-produced output may be authorless and thus lack copyright protection. This article, the first in a two-part series, analyses how each case deals with the question of authorship of computer-produced works and why the use of software diminishes copyright protection for a significant number of computer-generated works. The article critiques the application of conventional notions of human authorship developed in the pre-computer age to modern productions and suggests alternative approaches to authorship that satisfy both the major objectives of copyright policy and the need to adapt to the computer age. The article argues that, without a broader judicial approach to authorship of computer-generated works, Parliament must remedy the lacuna in protection for these ‘authorless’ works. Possible solutions for reform are suggested. In a forthcoming article, the author comprehensively examines those reform proposals
WHITE DWARF COSMOCHRONOLOGY IN THE SOLAR NEIGHBORHOOD
No full textThe study of the stellar formation history in the solar neighborhood is a powerful technique to recover information about the early stages and evolution of the Milky Way. We present a new method that consists of directly probing the formation history from the nearby stellar remnants. We rely on the volume complete sample of white dwarfs within 20 pc, where accurate cooling ages and masses have been determined. The well characterized initial-final mass relation is employed in order to recover the initial masses (1 less than or similar to M-initial/M-circle dot less than or similar to 8) and total ages for the local degenerate sample. We correct for moderate biases that are necessary to transform our results to a global stellar formation rate, which can be compared to similar studies based on the properties of main- sequence stars in the solar neighborhood. Our method provides precise formation rates for all ages except in very recent times, and the results suggest an enhanced formation rate for the solar neighborhood in the last 5 Gyr compared to the range 5 < Age (Gyr) < 10. Furthermore, the observed total age of similar to 10 Gyr for the oldest white dwarfs in the local sample is consistent with the early seminal studies that have determined the age of the Galactic disk from stellar remnants. The main shortcoming of our study is the small size of the local white dwarf sample. However, the presented technique can be applied to larger samples in the future
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