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  • Superfluidity and superconductivity  (161)
  • Astronomy  (142)
  • Models, Molecular
  • 2010-2014  (319)
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  • 1
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    Mutations in the profilin 1 gene cause familial amyotrophic lateral sclerosis (2012)
    Nature Publishing Group (NPG)
    Details
    Publication Date: 2012-07-18
    Description: Amyotrophic lateral sclerosis (ALS) is a late-onset neurodegenerative disorder resulting from motor neuron death. Approximately 10% of cases are familial (FALS), typically with a dominant inheritance mode. Despite numerous advances in recent years, nearly 50% of FALS cases have unknown genetic aetiology. Here we show that mutations within the profilin 1 (PFN1) gene can cause FALS. PFN1 is crucial for the conversion of monomeric (G)-actin to filamentous (F)-actin. Exome sequencing of two large ALS families showed different mutations within the PFN1 gene. Further sequence analysis identified 4 mutations in 7 out of 274 FALS cases. Cells expressing PFN1 mutants contain ubiquitinated, insoluble aggregates that in many cases contain the ALS-associated protein TDP-43. PFN1 mutants also display decreased bound actin levels and can inhibit axon outgrowth. Furthermore, primary motor neurons expressing mutant PFN1 display smaller growth cones with a reduced F/G-actin ratio. These observations further document that cytoskeletal pathway alterations contribute to ALS pathogenesis.〈br /〉〈br /〉〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3575525/" target="_blank"〉〈img src="https://static.pubmed.gov/portal/portal3rc.fcgi/4089621/img/3977009" border="0"〉〈/a〉   〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3575525/" target="_blank"〉This paper as free author manuscript - peer-reviewed and accepted for publication〈/a〉〈br /〉〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Wu, Chi-Hong -- Fallini, Claudia -- Ticozzi, Nicola -- Keagle, Pamela J -- Sapp, Peter C -- Piotrowska, Katarzyna -- Lowe, Patrick -- Koppers, Max -- McKenna-Yasek, Diane -- Baron, Desiree M -- Kost, Jason E -- Gonzalez-Perez, Paloma -- Fox, Andrew D -- Adams, Jenni -- Taroni, Franco -- Tiloca, Cinzia -- Leclerc, Ashley Lyn -- Chafe, Shawn C -- Mangroo, Dev -- Moore, Melissa J -- Zitzewitz, Jill A -- Xu, Zuo-Shang -- van den Berg, Leonard H -- Glass, Jonathan D -- Siciliano, Gabriele -- Cirulli, Elizabeth T -- Goldstein, David B -- Salachas, Francois -- Meininger, Vincent -- Rossoll, Wilfried -- Ratti, Antonia -- Gellera, Cinzia -- Bosco, Daryl A -- Bassell, Gary J -- Silani, Vincenzo -- Drory, Vivian E -- Brown, Robert H Jr -- Landers, John E -- 1R01NS050557/NS/NINDS NIH HHS/ -- 1R01NS065847/NS/NINDS NIH HHS/ -- R01 NS050557/NS/NINDS NIH HHS/ -- RC2 NS070342/NS/NINDS NIH HHS/ -- RC2-NS070-342/NS/NINDS NIH HHS/ -- T32 GM007754/GM/NIGMS NIH HHS/ -- U01 NS052225/NS/NINDS NIH HHS/ -- UL1 TR000454/TR/NCATS NIH HHS/ -- Howard Hughes Medical Institute/ -- England -- Nature. 2012 Aug 23;488(7412):499-503. doi: 10.1038/nature11280.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Department of Neurology, University of Massachusetts Medical School, Worcester, Massachusetts 01605, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/22801503" target="_blank"〉PubMed〈/a〉
    Keywords: Actins/metabolism ; Amino Acid Sequence ; Amyotrophic Lateral Sclerosis/diagnosis/*genetics/metabolism/*pathology ; Animals ; Axons/metabolism/pathology ; Cells, Cultured ; European Continental Ancestry Group/genetics ; Exome/genetics ; Female ; Genetic Predisposition to Disease/*genetics ; Growth Cones/metabolism ; High-Throughput Nucleotide Sequencing ; Humans ; Jews/genetics ; Male ; Mice ; Models, Molecular ; Molecular Sequence Data ; Motor Neurons/cytology/metabolism ; Mutant Proteins/genetics/*metabolism ; Mutation/*genetics ; Pedigree ; Profilins/*genetics/*metabolism ; Protein Conformation ; Ubiquitination
    Print ISSN: 0028-0836
    Electronic ISSN: 1476-4687
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
    Published by Nature Publishing Group (NPG)
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  • 2
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    Medulloblastoma exome sequencing uncovers subtype-specific somatic mutations (2012)
    Nature Publishing Group (NPG)
    Details
    Publication Date: 2012-07-24
    Description: Medulloblastomas are the most common malignant brain tumours in children. Identifying and understanding the genetic events that drive these tumours is critical for the development of more effective diagnostic, prognostic and therapeutic strategies. Recently, our group and others described distinct molecular subtypes of medulloblastoma on the basis of transcriptional and copy number profiles. Here we use whole-exome hybrid capture and deep sequencing to identify somatic mutations across the coding regions of 92 primary medulloblastoma/normal pairs. Overall, medulloblastomas have low mutation rates consistent with other paediatric tumours, with a median of 0.35 non-silent mutations per megabase. We identified twelve genes mutated at statistically significant frequencies, including previously known mutated genes in medulloblastoma such as CTNNB1, PTCH1, MLL2, SMARCA4 and TP53. Recurrent somatic mutations were newly identified in an RNA helicase gene, DDX3X, often concurrent with CTNNB1 mutations, and in the nuclear co-repressor (N-CoR) complex genes GPS2, BCOR and LDB1. We show that mutant DDX3X potentiates transactivation of a TCF promoter and enhances cell viability in combination with mutant, but not wild-type, beta-catenin. Together, our study reveals the alteration of WNT, hedgehog, histone methyltransferase and now N-CoR pathways across medulloblastomas and within specific subtypes of this disease, and nominates the RNA helicase DDX3X as a component of pathogenic beta-catenin signalling in medulloblastoma.〈br /〉〈br /〉〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3413789/" target="_blank"〉〈img src="https://static.pubmed.gov/portal/portal3rc.fcgi/4089621/img/3977009" border="0"〉〈/a〉   〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3413789/" target="_blank"〉This paper as free author manuscript - peer-reviewed and accepted for publication〈/a〉〈br /〉〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Pugh, Trevor J -- Weeraratne, Shyamal Dilhan -- Archer, Tenley C -- Pomeranz Krummel, Daniel A -- Auclair, Daniel -- Bochicchio, James -- Carneiro, Mauricio O -- Carter, Scott L -- Cibulskis, Kristian -- Erlich, Rachel L -- Greulich, Heidi -- Lawrence, Michael S -- Lennon, Niall J -- McKenna, Aaron -- Meldrim, James -- Ramos, Alex H -- Ross, Michael G -- Russ, Carsten -- Shefler, Erica -- Sivachenko, Andrey -- Sogoloff, Brian -- Stojanov, Petar -- Tamayo, Pablo -- Mesirov, Jill P -- Amani, Vladimir -- Teider, Natalia -- Sengupta, Soma -- Francois, Jessica Pierre -- Northcott, Paul A -- Taylor, Michael D -- Yu, Furong -- Crabtree, Gerald R -- Kautzman, Amanda G -- Gabriel, Stacey B -- Getz, Gad -- Jager, Natalie -- Jones, David T W -- Lichter, Peter -- Pfister, Stefan M -- Roberts, Thomas M -- Meyerson, Matthew -- Pomeroy, Scott L -- Cho, Yoon-Jae -- CA050661/CA/NCI NIH HHS/ -- L40 NS063706/NS/NINDS NIH HHS/ -- P30 HD018655/HD/NICHD NIH HHS/ -- P30 HD18655/HD/NICHD NIH HHS/ -- R01 CA030002/CA/NCI NIH HHS/ -- R01 CA105607/CA/NCI NIH HHS/ -- R01 CA109467/CA/NCI NIH HHS/ -- R01 CA148699/CA/NCI NIH HHS/ -- R01 CA154480/CA/NCI NIH HHS/ -- R01 NS046789/NS/NINDS NIH HHS/ -- R01CA105607/CA/NCI NIH HHS/ -- R01CA109467/CA/NCI NIH HHS/ -- R01CA148699/CA/NCI NIH HHS/ -- R25 NS070682/NS/NINDS NIH HHS/ -- R25NS070682/NS/NINDS NIH HHS/ -- U54 HG003067/HG/NHGRI NIH HHS/ -- U54HG003067/HG/NHGRI NIH HHS/ -- Canadian Institutes of Health Research/Canada -- Howard Hughes Medical Institute/ -- England -- Nature. 2012 Aug 2;488(7409):106-10. doi: 10.1038/nature11329.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Broad Institute of MIT and Harvard, Cambridge, Massachusetts 02142, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/22820256" target="_blank"〉PubMed〈/a〉
    Keywords: Cerebellar Neoplasms/classification/*genetics ; Child ; DEAD-box RNA Helicases/chemistry/genetics/metabolism ; DNA Helicases/chemistry/genetics ; DNA-Binding Proteins/genetics ; Exome/*genetics ; Genome, Human/*genetics ; Hedgehog Proteins/metabolism ; Histone-Lysine N-Methyltransferase/genetics/metabolism ; Humans ; Intracellular Signaling Peptides and Proteins/genetics ; LIM Domain Proteins/genetics ; Medulloblastoma/classification/*genetics ; Models, Molecular ; Mutation/*genetics ; Neoplasm Proteins/genetics ; Nuclear Proteins/chemistry/genetics ; Promoter Regions, Genetic/genetics ; Protein Structure, Tertiary/genetics ; Proto-Oncogene Proteins/genetics ; Receptors, Cell Surface/genetics ; Repressor Proteins/genetics ; Signal Transduction ; TCF Transcription Factors/metabolism ; Transcription Factors/chemistry/genetics ; Tumor Suppressor Protein p53/genetics ; Wnt Proteins/metabolism ; beta Catenin/genetics/metabolism
    Print ISSN: 0028-0836
    Electronic ISSN: 1476-4687
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
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  • 3
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    Development of Kilo-Pixel Arrays of Transition-Edge Sensors for X-Ray Spectroscopy (2012)
    In:  CASI
    Details
    Publication Date: 2019-07-19
    Description: We are developing kilo-pixel arrays of transition-edge sensor (TES) microcalorimeters for future X-ray astronomy observatories or for use in laboratory astrophysics applications. For example, Athena/XMS (currently under study by the european space agency) would require a close-packed 32x32 pixel array on a 250-micron pitch with 〈 3.0 eV full-width-half-maximum energy resolution at 6 keV and at count-rates of up to 50 counts/pixel/second. We present characterization of 32x32 arrays. These detectors will be readout using state of the art SQUID based time-domain multiplexing (TDM). We will also present the latest results in integrating these detectors and the TDM readout technology into a 16 row x N column field-able instrument.
    Keywords: Astronomy
    Type: GSFC.ABS.01034.2012 , Applied Superconductivity Conference; Oct 07, 2012 - Oct 11, 2012; Portland, OR; United States
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  • 4
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    Mutations in kelch-like 3 and cullin 3 cause hypertension and electrolyte abnormalities (2012)
    Nature Publishing Group (NPG)
    Details
    Publication Date: 2012-01-24
    Description: Hypertension affects one billion people and is a principal reversible risk factor for cardiovascular disease. Pseudohypoaldosteronism type II (PHAII), a rare Mendelian syndrome featuring hypertension, hyperkalaemia and metabolic acidosis, has revealed previously unrecognized physiology orchestrating the balance between renal salt reabsorption and K(+) and H(+) excretion. Here we used exome sequencing to identify mutations in kelch-like 3 (KLHL3) or cullin 3 (CUL3) in PHAII patients from 41 unrelated families. KLHL3 mutations are either recessive or dominant, whereas CUL3 mutations are dominant and predominantly de novo. CUL3 and BTB-domain-containing kelch proteins such as KLHL3 are components of cullin-RING E3 ligase complexes that ubiquitinate substrates bound to kelch propeller domains. Dominant KLHL3 mutations are clustered in short segments within the kelch propeller and BTB domains implicated in substrate and cullin binding, respectively. Diverse CUL3 mutations all result in skipping of exon 9, producing an in-frame deletion. Because dominant KLHL3 and CUL3 mutations both phenocopy recessive loss-of-function KLHL3 mutations, they may abrogate ubiquitination of KLHL3 substrates. Disease features are reversed by thiazide diuretics, which inhibit the Na-Cl cotransporter in the distal nephron of the kidney; KLHL3 and CUL3 are expressed in this location, suggesting a mechanistic link between KLHL3 and CUL3 mutations, increased Na-Cl reabsorption, and disease pathogenesis. These findings demonstrate the utility of exome sequencing in disease gene identification despite the combined complexities of locus heterogeneity, mixed models of transmission and frequent de novo mutation, and establish a fundamental role for KLHL3 and CUL3 in blood pressure, K(+) and pH homeostasis.〈br /〉〈br /〉〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3278668/" target="_blank"〉〈img src="https://static.pubmed.gov/portal/portal3rc.fcgi/4089621/img/3977009" border="0"〉〈/a〉   〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3278668/" target="_blank"〉This paper as free author manuscript - peer-reviewed and accepted for publication〈/a〉〈br /〉〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Boyden, Lynn M -- Choi, Murim -- Choate, Keith A -- Nelson-Williams, Carol J -- Farhi, Anita -- Toka, Hakan R -- Tikhonova, Irina R -- Bjornson, Robert -- Mane, Shrikant M -- Colussi, Giacomo -- Lebel, Marcel -- Gordon, Richard D -- Semmekrot, Ben A -- Poujol, Alain -- Valimaki, Matti J -- De Ferrari, Maria E -- Sanjad, Sami A -- Gutkin, Michael -- Karet, Fiona E -- Tucci, Joseph R -- Stockigt, Jim R -- Keppler-Noreuil, Kim M -- Porter, Craig C -- Anand, Sudhir K -- Whiteford, Margo L -- Davis, Ira D -- Dewar, Stephanie B -- Bettinelli, Alberto -- Fadrowski, Jeffrey J -- Belsha, Craig W -- Hunley, Tracy E -- Nelson, Raoul D -- Trachtman, Howard -- Cole, Trevor R P -- Pinsk, Maury -- Bockenhauer, Detlef -- Shenoy, Mohan -- Vaidyanathan, Priya -- Foreman, John W -- Rasoulpour, Majid -- Thameem, Farook -- Al-Shahrouri, Hania Z -- Radhakrishnan, Jai -- Gharavi, Ali G -- Goilav, Beatrice -- Lifton, Richard P -- KL2 RR024138/RR/NCRR NIH HHS/ -- KL2 RR024138-07/RR/NCRR NIH HHS/ -- P30 DK079310/DK/NIDDK NIH HHS/ -- P30 DK079310-04S1/DK/NIDDK NIH HHS/ -- P30-DK079310/DK/NIDDK NIH HHS/ -- UL1-RR024139/RR/NCRR NIH HHS/ -- Howard Hughes Medical Institute/ -- England -- Nature. 2012 Jan 22;482(7383):98-102. doi: 10.1038/nature10814.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Department of Genetics and Howard Hughes Medical Institute, Yale University School of Medicine, New Haven, Connecticut 06510, USA.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/22266938" target="_blank"〉PubMed〈/a〉
    Keywords: Amino Acid Sequence ; Animals ; Base Sequence ; Blood Pressure/genetics ; Carrier Proteins/chemistry/*genetics ; Cohort Studies ; Cullin Proteins/chemistry/*genetics ; Electrolytes ; Exons/genetics ; Female ; Gene Expression Profiling ; Genes, Dominant/genetics ; Genes, Recessive/genetics ; Genotype ; Homeostasis/genetics ; Humans ; Hydrogen-Ion Concentration ; Hypertension/complications/*genetics/physiopathology ; Male ; Mice ; Models, Molecular ; Molecular Sequence Data ; Mutation/*genetics ; Phenotype ; Potassium/metabolism ; Pseudohypoaldosteronism/complications/*genetics/physiopathology ; Sodium Chloride/metabolism ; Water-Electrolyte Imbalance/complications/*genetics/physiopathology
    Print ISSN: 0028-0836
    Electronic ISSN: 1476-4687
    Topics: Biology , Chemistry and Pharmacology , Medicine , Natural Sciences in General , Physics
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  • 5
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    Natively inhibited Trypanosoma brucei cathepsin B structure determined by using an X-ray laser (2012)
    American Association for the Advancement of Science (AAAS)
    Details
    Publication Date: 2012-12-01
    Description: The Trypanosoma brucei cysteine protease cathepsin B (TbCatB), which is involved in host protein degradation, is a promising target to develop new treatments against sleeping sickness, a fatal disease caused by this protozoan parasite. The structure of the mature, active form of TbCatB has so far not provided sufficient information for the design of a safe and specific drug against T. brucei. By combining two recent innovations, in vivo crystallization and serial femtosecond crystallography, we obtained the room-temperature 2.1 angstrom resolution structure of the fully glycosylated precursor complex of TbCatB. The structure reveals the mechanism of native TbCatB inhibition and demonstrates that new biomolecular information can be obtained by the "diffraction-before-destruction" approach of x-ray free-electron lasers from hundreds of thousands of individual microcrystals.〈br /〉〈br /〉〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3786669/" target="_blank"〉〈img src="https://static.pubmed.gov/portal/portal3rc.fcgi/4089621/img/3977009" border="0"〉〈/a〉   〈a href="https://www.ncbi.nlm.nih.gov/pmc/articles/PMC3786669/" target="_blank"〉This paper as free author manuscript - peer-reviewed and accepted for publication〈/a〉〈br /〉〈br /〉〈span class="detail_caption"〉Notes: 〈/span〉Redecke, Lars -- Nass, Karol -- DePonte, Daniel P -- White, Thomas A -- Rehders, Dirk -- Barty, Anton -- Stellato, Francesco -- Liang, Mengning -- Barends, Thomas R M -- Boutet, Sebastien -- Williams, Garth J -- Messerschmidt, Marc -- Seibert, M Marvin -- Aquila, Andrew -- Arnlund, David -- Bajt, Sasa -- Barth, Torsten -- Bogan, Michael J -- Caleman, Carl -- Chao, Tzu-Chiao -- Doak, R Bruce -- Fleckenstein, Holger -- Frank, Matthias -- Fromme, Raimund -- Galli, Lorenzo -- Grotjohann, Ingo -- Hunter, Mark S -- Johansson, Linda C -- Kassemeyer, Stephan -- Katona, Gergely -- Kirian, Richard A -- Koopmann, Rudolf -- Kupitz, Chris -- Lomb, Lukas -- Martin, Andrew V -- Mogk, Stefan -- Neutze, Richard -- Shoeman, Robert L -- Steinbrener, Jan -- Timneanu, Nicusor -- Wang, Dingjie -- Weierstall, Uwe -- Zatsepin, Nadia A -- Spence, John C H -- Fromme, Petra -- Schlichting, Ilme -- Duszenko, Michael -- Betzel, Christian -- Chapman, Henry N -- 1R01GM095583/GM/NIGMS NIH HHS/ -- R01 GM095583/GM/NIGMS NIH HHS/ -- U54 GM094599/GM/NIGMS NIH HHS/ -- New York, N.Y. -- Science. 2013 Jan 11;339(6116):227-30. doi: 10.1126/science.1229663. Epub 2012 Nov 29.〈br /〉〈span class="detail_caption"〉Author address: 〈/span〉Joint Laboratory for Structural Biology of Infection and Inflammation, Institute of Biochemistry and Molecular Biology, University of Hamburg, and Institute of Biochemistry, University of Lubeck, at Deutsches Elektronen-Synchrotron, Notkestrasse 85, 22607 Hamburg, Germany.〈br /〉〈span class="detail_caption"〉Record origin:〈/span〉 〈a href="http://www.ncbi.nlm.nih.gov/pubmed/23196907" target="_blank"〉PubMed〈/a〉
    Keywords: Amino Acid Sequence ; Animals ; Catalytic Domain ; Cathepsin B/antagonists & inhibitors/*chemistry ; Crystallization ; Crystallography, X-Ray ; Enzyme Precursors/chemistry ; Glycosylation ; Models, Molecular ; Molecular Sequence Data ; Protein Conformation ; Protozoan Proteins/antagonists & inhibitors/*chemistry ; Sf9 Cells ; Spodoptera ; Trypanosoma brucei brucei/*enzymology ; X-Rays
    Print ISSN: 0036-8075
    Electronic ISSN: 1095-9203
    Topics: Biology , Chemistry and Pharmacology , Computer Science , Medicine , Natural Sciences in General , Physics
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  • 6
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    The XMM-Newton Survey of the Small Magellanic Cloud (2012)
    In:  CASI
    Details
    Publication Date: 2019-07-13
    Description: Context. Although numerous archival XMM-Newton observations existed towards the Small Magellanic Cloud (SMC) before 2009, only a fraction of the whole galaxy had been covered. Aims. Between May 2009 and March 2010, we carried out an XMM-Newton survey of the SMC, to ensure a complete coverage of both its bar and wing. Thirty-three observations of 30 different fields with a total exposure of about one Ms filled the previously missing parts. Methods. We systematically processed all available SMC data from the European Photon Imaging Camera. After rejecting observations with very high background, we included 53 archival and the 33 survey observations. We produced images in five different energy bands. We applied astrometric boresight corrections using secure identifications of X-ray sources and combined all the images to produce a mosaic covering the main body of the SMC. Results. We present an overview of the XMM-Newton observations, describe their analysis, and summarize our first results, which will be presented in detail in follow-up papers. Here, we mainly focus on extended X-ray sources, such as supernova remnants (SNRs) and clusters of galaxies, that are seen in our X-ray images. Conclusions. Our XMM-Newton survey represents the deepest complete survey of the SMC in the 0.1512.0 keV X-ray band. We propose three new SNRs that have low surface brightnesses of a few 1014 erg cm2 s1 arcmin2 and large extents. In addition, several known remnants appear larger than previously measured at either X-rays or other wavelengths extending the size distribution of SMC SNRs to larger values.
    Keywords: Astronomy
    Type: GSFC-E-DAA-TN9857 , Astronomy & Astrophysics; 545; A128
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  • 7
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    Simultaneous Planck, Swift, and Fermi Observations of X-ray and Gamma-ray Selected Blazars (2012)
    In:  CASI
    Details
    Publication Date: 2019-07-12
    Description: We present simultaneous Planck, Swift, Fermi, and ground-based data for 105 blazars belonging to three samples with flux limits in the soft X-ray, hard X-ray, and gamma-ray bands, with additional 5 GHz flux-density limits to ensure a good probability of a Planck detection. We compare our results to those of a companion paper presenting simultaneous Planck and multi-frequency observations of 104 radio-loud northern active galactic nuclei selected at radio frequencies. While we confirm several previous results, our unique data set allows us to demonstrate that the selection method strongly influences the results, producing biases that cannot be ignored. Almost all the BL Lac objects have been detected by the Fermi Large Area Telescope (LAT), whereas 30% to 40% of the flat-spectrum radio quasars (FSRQs) in the radio, soft X-ray, and hard X-ray selected samples are still below the gamma-ray detection limit even after integrating 27 months of Fermi-LAT data. The radio to sub-millimetre spectral slope of blazars is quite flat, with (alpha) approx 0 up to about 70GHz, above which it steepens to (alpha) approx -0.65. The BL Lacs have significantly flatter spectra than FSRQs at higher frequencies. The distribution of the rest-frame synchrotron peak frequency (nu(sup s)(sub peak)) in the spectral energy distribution (SED) of FSRQs is the same in all the blazar samples with (nu(sup s)(sub peak)) = 10(exp 13.1 +/- 0.1) Hz, while the mean inverse Compton peak frequency, (nu(sup IC)(sub peak)), ranges from 10(exp 21) to 10(exp 22) Hz. The distributions of nu(sup s)(sub peak) and nu(sup IC)(sub peak) of BL Lacs are much broader and are shifted to higher energies than those of FSRQs; their shapes strongly depend on the selection method. The Compton dominance of blazars. defined as the ratio of the inverse Compton to synchrotron peak luminosities, ranges from less than 0.2 to nearly 100, with only FSRQs reaching values larger than about 3. Its distribution is broad and depends strongly on the selection method, with gamma-ray selected blazars peaking at approx 7 or more, and radio-selected blazars at values close to 1, thus implying that the common assumption that the blazar power budget is largely dominated by high-energy emission is a selection effect. A comparison of our multi-frequency data with theoretical predictions shows that simple homogeneous SSC models cannot explain the simultaneous SEDs of most of the gamma-ray detected blazars in all samples. The SED of the blazars that were not detected by Fermi~LAT may instead be consistent with SSC emission. Our data challenge the correlation between bolometric luminosity and nu(sup s)(sub peak) predicted by the blazar sequence.
    Keywords: Astronomy
    Type: GSFC.JA.7378.2012
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  • 8
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    The Fermi Large Area Telescope on Orbit: Event Classification, Instrument Response Functions, and Calibration (2012)
    In:  CASI
    Details
    Publication Date: 2019-07-12
    Description: The Fermi Large Area Telescope (Fermi-LAT, hereafter LAT), the primary instrument on the Fermi Gamma-ray Space Telescope (Fermi) mission, is an imaging, wide field-of-view, high-energy -ray telescope, covering the energy range from 20 MeV to more than 300 GeV. During the first years of the mission the LAT team has gained considerable insight into the in-flight performance of the instrument. Accordingly, we have updated the analysis used to reduce LAT data for public release as well as the Instrument Response Functions (IRFs), the description of the instrument performance provided for data analysis. In this paper we describe the effects that motivated these updates. Furthermore, we discuss how we originally derived IRFs from Monte Carlo simulations and later corrected those IRFs for discrepancies observed between flight and simulated data. We also give details of the validations performed using flight data and quantify the residual uncertainties in the IRFs. Finally, we describe techniques the LAT team has developed to propagate those uncertainties into estimates of the systematic errors on common measurements such as fluxes and spectra of astrophysical sources.
    Keywords: Astronomy
    Type: GSFC.JA.7125.2012
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  • 9
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    Antiferromagnetic spin fluctuations in the heavy-fermion superconductor Ce_{2}PdIn_{8} (2012)
    American Physical Society (APS)
    Details
    Publication Date: 2012-09-28
    Description: Author(s): V. H. Tran, A. D. Hillier, D. T. Adroja, and D. Kaczorowski Inelastic neutron scattering and muon spin relaxation/rotation ( μ SR) measurements were performed on the heavy-fermion superconductor Ce 2 PdIn 8 . The observed scaling of the imaginary part of the dynamical susceptibility χ ′′ T α ∝ f ( ℏ ω / k B T ) with α =3/2 revealed a non-Fermi liquid character of the normal sta... [Phys. Rev. B 86, 094525] Published Thu Sep 27, 2012
    Keywords: Superfluidity and superconductivity
    Print ISSN: 1098-0121
    Electronic ISSN: 1095-3795
    Topics: Physics
    Published by American Physical Society (APS)
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  • 10
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    Slow order-parameter fluctuations in superconducting Pb and Ag/Nb films observed using β-detected nuclear magnetic resonance (2012)
    American Physical Society (APS)
    Details
    Publication Date: 2012-06-05
    Description: Author(s): E. Morenzoni, H. Saadaoui, D. Wang, M. Horisberger, E. C. Kirk, W. A. MacFarlane, G. D. Morris, K. H. Chow, M. D. Hossain, C. P. Levy, T. J. Parolin, M. R. Pearson, Q. Song, and R. F. Kiefl We report β -NMR investigations of polarized 8 Li implanted in thin Pb and Ag/Nb films. At the critical superconducting temperature, we observe a singular peak in the spin relaxation rate in small longitudinal magnetic fields, which is attributed to unexpected slow fluctuations in the superconducting ... [Phys. Rev. B 85, 220501] Published Mon Jun 04, 2012
    Keywords: Superfluidity and superconductivity
    Print ISSN: 1098-0121
    Electronic ISSN: 1095-3795
    Topics: Physics
    Published by American Physical Society (APS)
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