933 research outputs found

    Subunit Stoichiometry of a Heteromultimeric G protein-coupled Inward-rectifier K^+ Channel

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    We investigated the stoichiometry of the heteromultimeric G protein-coupled inward-recitfier K^+ channel (GIRK) formed from GIRK1 and GIRK4 subunits. Multimeric GIRK constructs with several concatenated channel subunits were expressed in Xenopus oocytes. Coexpression of various trimeric constructs with different monomers clearly showed that the functional channel has stoichiometry (GIRK1)_2(GIRK4)_2. Efforts to establish a preferred arrangement of subunits around the channel pore suggest that more than one arrangement may be viable

    A Chromosomal Deletion and New Frameshift Mutation Cause ARSACS in an African-American

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    Autosomal Recessive Spastic Ataxia of Charlevoix-Saguenay (ARSACS) is a rare, progressive, neurodegenerative disease characterized by ataxia, spasticity and polyneuropathy. First described in the French-Canadian population of Quebec in 1978, ARSACS has since been identified in multiple patients worldwide. In this clinical case report, we describe the evaluation of an 11-years-old African-American male who presented to neuromuscular clinic for assessment of a gait abnormality. He had a history of gross motor delay since early childhood, frequent falls and a below average IQ. Chromosomal microarray revealed a 1.422 megabase loss in the 13q12.12 region, which includes the SACS gene. Next Generation Sequencing then showed a novel, predicted to be pathogenic missense mutation (c.11824dup) of this gene. His clinical presentation and neurological imaging further confirmed the diagnosis of ARSACS. To our knowledge, this is the first reported case of this disease in the African-American population of the United States. This case report further highlights the growing trend of identifying genetic diseases previously restricted to single, ethnically isolated regions in many different ethnic groups worldwide

    Conformational effects on high-spin organic molecules

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    The ability of m-phenylene to ferromagnetically couple spin-containing substituents that are substantially twisted out of conjugation is investigated. The "bis(TMM)" strategy is employed, in which two triplet TMM biradicals are linked through m-phenylene to produce relatively stable, organic tetraradicals that are characterized by EPR spectroscopy. Under conditions of moderate twisting (4), ferromagnetic coupling is seen, and the tetraradical has a quintet ground state. Severely twisting both TMMs as in 13 disrupts spin communication, and two noninteracting triplets are produced. This is in contrast to other highly twisted m-phenylene derivatives, in which antiferromagnetic coupling has been observed. Surprisingly, severely twisting only one TMM (14) still produces ferromagnetic coupling and a quintet ground state through a spin polarization mechanism analogous to that proposed for 90° twisted ethylene. Several ring-constrained TMMs (17-19) are investigated as models for more nearly planar systems

    Methyl and t-butyl reorientation in an organic molecular solid

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    We have determined the molecular and crystal structure of 4,5-dibromo-2,7-di-t-butyl-9,9-dimethylxanthene and measured the 1H spin–lattice relaxation rate from 87 to 270 K at NMR frequencies of ω/2π=8.50, 22.5, and 53.0 MHz. All molecules in the crystal see the same intra and intermolecular environment and the repeating unit is half a molecule. We have extended models developed for 1H spin–lattice relaxation resulting from the reorientation of a t-butyl group and its constituent methyl groups to include these rotors and the 9-methyl groups. The relaxation rate data is well-fitted assuming that the t-butyl groups and all three of their constituent methyl groups, as well as the 9-methyl groups all reorient with an NMR activation energy of 15.8±1.6 kJ mol−1 corresponding to a barrier of 17.4±3.2 kJ mol−1. Only intramethyl and intra-t-butyl intermethyl spin–spin interactions need be considered. A unique random-motion Debye (or BPP) spectral density will not fit the data for any reasonable choice of parameters. A distribution of activation energies is required

    Methyl and t-butyl reorientation in an organic molecular solid

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    We have determined the molecular and crystal structure of 4,5-dibromo-2,7-di-t-butyl-9,9-dimethylxanthene and measured the 1H spin–lattice relaxation rate from 87 to 270 K at NMR frequencies of ω/2π=8.50, 22.5, and 53.0 MHz. All molecules in the crystal see the same intra and intermolecular environment and the repeating unit is half a molecule. We have extended models developed for 1H spin–lattice relaxation resulting from the reorientation of a t-butyl group and its constituent methyl groups to include these rotors and the 9-methyl groups. The relaxation rate data is well-fitted assuming that the t-butyl groups and all three of their constituent methyl groups, as well as the 9-methyl groups all reorient with an NMR activation energy of 15.8±1.6 kJ mol−1 corresponding to a barrier of 17.4±3.2 kJ mol−1. Only intramethyl and intra-t-butyl intermethyl spin–spin interactions need be considered. A unique random-motion Debye (or BPP) spectral density will not fit the data for any reasonable choice of parameters. A distribution of activation energies is required

    Workload and Performance in FOPA: A Strategic Planning Interface for Air Traffic Control

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    The Flight Organizer Planning Aid (FOPA) interface was designed to aid strategic planning in air traffic control. In particular, FOPA was designed to address difficulties in solving en route sequencing problems (combining multiple streams of traffic into a single stream heading for a destination). We compared the planning performance of 12 full performance level air traffic controllers using either the FOPA interface or normal flight progress strips. Planning performance was significantly better when using FOPA; subjective workload was also reduced. The results indicate that a key advantage of FOPA lies in its dynamic linkage between the flight organizer screen where aircraft tokens can be categorized into color-coded blocks and their matching spatial representation on the radar.Yeshttps://us.sagepub.com/en-us/nam/manuscript-submission-guideline

    Conformational effects on high-spin organic molecules

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    The ability of m-phenylene to ferromagnetically couple spin-containing substituents that are substantially twisted out of conjugation is investigated. The "bis(TMM)" strategy is employed, in which two triplet TMM biradicals are linked through m-phenylene to produce relatively stable, organic tetraradicals that are characterized by EPR spectroscopy. Under conditions of moderate twisting (4), ferromagnetic coupling is seen, and the tetraradical has a quintet ground state. Severely twisting both TMMs as in 13 disrupts spin communication, and two noninteracting triplets are produced. This is in contrast to other highly twisted m-phenylene derivatives, in which antiferromagnetic coupling has been observed. Surprisingly, severely twisting only one TMM (14) still produces ferromagnetic coupling and a quintet ground state through a spin polarization mechanism analogous to that proposed for 90° twisted ethylene. Several ring-constrained TMMs (17-19) are investigated as models for more nearly planar systems

    Nonexponential Solid State 1H and 19F Spin–Lattice Relaxation, Single-crystal X-ray Diffraction, and Isolated-Molecule and Cluster Electronic Structure Calculations in an Organic Solid: Coupled Methyl Group Rotation and Methoxy Group Libration in 4,4′-Dimethoxyoctafluorobiphenyl

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    We investigate the relationship between intramolecular rotational dynamics and molecular and crystal structure in 4,4′-dimethoxyoctafluorobiphenyl. The techniques are electronic structure calculations, X-ray diffractometry, and 1H and 19F solid state nuclear magnetic resonance relaxation. We compute and measure barriers for coupled methyl group rotation and methoxy group libration. We compare the structure and the structure-motion relationship in 4,4′-dimethoxyoctafluorobiphenyl with the structure and the structure-motion relationship in related compounds in order to observe trends concerning the competition between intramolecular and intermolecular interactions. The 1H spin–lattice relaxation is nonexponential in both the high-temperature short-correlation time limit and in the low-temperature long-correlation time limit, albeit for different reasons. The 19F spin–lattice relaxation is nonexponential at low temperatures and it is exponential at high temperatures

    A multiwavelength study of the remnant of nova GK Persei

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    We present new observations of the nebular remnant of the old nova GK Persei 1901, in the optical using the 2m HCT and at low radio frequencies using the GMRT. The evolution of the nova remnant indicates shock interaction with the ambient medium, especially in the southwest quadrant. Application of a simple model for the shock and its evolution to determine the time dependence of the radius of the shell in the southwest quadrant indicates that the shell is now expanding into an ambient medium that has a lower density compared to the density of the ambient medium ahead of the shock in 1987.There are indications of a recent interaction of the nova remnant with the ambient medium in the northeast quadrant also. The nova remnant of GK Per is detected at all the observed radio frequencies and is of similar extent as the optical remnant. Putting together our radio observations with VLA archival data on GK Per from 1997, we obtain three interesting results: 1. The spectrum above 1.4 GHz follows a power law with an index -0.7 and below 1.4 GHz follows a power law with an index ~ -0.85. This could be due to the presence of at least two populations of electrons dominating the global emission at different frequencies. 2. We record an annual secular decrease of 2.1% in the flux density of the nova remnant at 1.4 and 4.9 GHz between 1984 and 1997 which has left the spectral index unchanged at -0.7. No such decrease is observed in the flux densities below 1 GHz. 3. We record an increase in the flux density at 0.33 GHz compared to the previous estimate in 1987. We conclude that the remnant of nova GK Per is similar to supernova remnants and in particular, to the young supernova remnant Cas A.Comment: 10 pages; uses A&A style; figures 1, 2 & 6 are in JPEG format. Accepted for publication in Astronomy & Astrophysics. Full paper including Figures 1, 2 & 6 may be downloaded from http://www.iiap.res.in/personnel/gca/gca.htm
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