323 research outputs found
A New Photomechanical Molecular Switch Based on a Linear {\pi}-Conjugated System
We report the electronic transport properties of a new photo-addressable
molecular switch. The switching process relies on a new concept based on linear
{\pi}-conjugated dynamic systems, in which the geometry and hence the
electronic properties of an oligothiophene chain can be reversibly modified by
the photochemical trans-cis isomerization of an azobenzene unit fixed in a
lateral loop. Electron transport measurements through self-assembled monolayers
on gold, contacted with eGaIn top contact, show switching with a conductance
ratio up to 1E3. Ab initio calculations have been used to identify the most
energetically stable conformations of the molecular switch, the corresponding
calculated conductances qualitatively explain the trend observed in the
photo-switching experiments.Comment: Full manuscript and supporting information, J. Phys. Chem.
C,published on line (2017
A New Photomechanical Molecular Switch Based on a Linear {\pi}-Conjugated System
We report the electronic transport properties of a new photo-addressable
molecular switch. The switching process relies on a new concept based on linear
{\pi}-conjugated dynamic systems, in which the geometry and hence the
electronic properties of an oligothiophene chain can be reversibly modified by
the photochemical trans-cis isomerization of an azobenzene unit fixed in a
lateral loop. Electron transport measurements through self-assembled monolayers
on gold, contacted with eGaIn top contact, show switching with a conductance
ratio up to 1E3. Ab initio calculations have been used to identify the most
energetically stable conformations of the molecular switch, the corresponding
calculated conductances qualitatively explain the trend observed in the
photo-switching experiments.Comment: Full manuscript and supporting information, J. Phys. Chem.
C,published on line (2017
Clinical and genetic analysis of 29 Brazilian patients with Huntington’s disease-like phenotype
Huntington’s disease (HD) is a neurodegenerative disorder characterized by chorea,
behavioral disturbances and dementia, caused by a pathological expansion of the CAG
trinucleotide in the HTT gene. Several patients have been recognized with the typical HD
phenotype without the expected mutation. The objective of this study was to assess the
occurrence of diseases such as Huntington’s disease-like 2 (HDL2), spinocerebellar ataxia
(SCA) 1, SCA2, SCA3, SCA7, dentatorubral-pallidoluysian atrophy (DRPLA) and choreaacanthocytosis
(ChAc) among 29 Brazilian patients with a HD-like phenotype. In the group
analyzed, we found 3 patients with HDL2 and 2 patients with ChAc. The diagnosis was not
reached in 79.3% of the patients. HDL2 was the main cause of the HD-like phenotype in
the group analyzed, and is attributable to the African ancestry of this population. However,
the etiology of the disease remains undetermined in the majority of the HD negative
patients with HD-like phenotype.
Key words: Huntington’s disease, Huntington’s disease-like, chorea-acanthocytosis,
Huntington’s disease-like 2
Conductance statistics from a large array of sub-10 nm molecular junctions
Devices made of few molecules constitute the miniaturization limit that both
inorganic and organic-based electronics aspire to reach. However, integration
of millions of molecular junctions with less than 100 molecules each has been a
long technological challenge requiring well controlled nanometric electrodes.
Here we report molecular junctions fabricated on a large array of sub-10 nm
single crystal Au nanodots electrodes, a new approach that allows us to measure
the conductance of up to a million of junctions in a single conducting Atomic
Force Microscope (C-AFM) image. We observe two peaks of conductance for
alkylthiol molecules. Tunneling decay constant (beta) for alkanethiols, is in
the same range as previous studies. Energy position of molecular orbitals,
obtained by transient voltage spectroscopy, varies from peak to peak, in
correlation with conductance values.Comment: ACS Nano (in press
Mutation update for the GPC3 gene involved in Simpson-Golabi-Behmel syndrome and review of the literature
Simpson-Golabi-Behmel syndrome (SGBS) is an X-linked multiple congenital anomalies and overgrowth syndrome caused by a defect in the glypican-3 gene (GPC3). Until now, GPC3 mutations have been reported in isolated cases or small series and the global genotypic spectrum of these mutations has never been delineated. In this study, we review the 57 previously described GPC3 mutations and significantly expand this mutational spectrum with the description of 29 novel mutations. Compiling our data and those of the literature, we provide an overview of 86 distinct GPC3 mutations identified in 120 unrelated families, ranging from single nucleotide variations to complex genomic rearrangements and dispersed throughout the entire coding region of GPC3. The vast majority of them are deletions or truncating mutations (frameshift, nonsense mutations) predicted to result in a loss-of-function. Missense mutations are rare and the two which were functionally characterized, impaired GPC3 function by preventing GPC3 cleavage and cell surface addressing respectively. This report by describing for the first time the wide mutational spectrum of GPC3 could help clinicians and geneticists in interpreting GPC3 variants identified incidentally by high-throughput sequencing technologies and also reinforces the need for functional validation of non-truncating mutations (missense, in frame mutations, duplications)
Molecule-Electrode Interface Energetics in Molecular Junction: a Transition Voltage Spectroscopy Study
We assess the performances of the transition voltage spectroscopy (TVS)
method to determine the energies of the molecular orbitals involved in the
electronic transport though molecular junctions. A large number of various
molecular junctions made with alkyl chains but with different chemical
structure of the electrode-molecule interfaces are studied. In the case of
molecular junctions with clean, unoxidized electrode-molecule interfaces, i.e.
alkylthiols and alkenes directly grafted on Au and hydrogenated Si,
respectively, we measure transition voltages in the range 0.9 - 1.4 V. We
conclude that the TVS method allows estimating the onset of the tail of the
LUMO density of states, at energy located 1.0 - 1.2 eV above the electrode
Fermi energy. For oxidized interfaces (e.g. the same monolayer measured with Hg
or eGaIn drops, or monolayers formed on a slightly oxidized silicon substrate),
lower transition voltages (0.1 - 0.6 V) are systematically measured. These
values are explained by the presence of oxide-related density of states at
energies lower than the HOMO-LUMO of the molecules. As such, the TVS method is
a useful technique to assess the quality of the molecule-electrode interfaces
in molecular junctions.Comment: Accepted for publication in J. Phys. Chem C. One pdf file including
manuscript, figures and supporting informatio
The CAG repeat at the Huntington disease gene in the Portuguese population : insights into its dynamics and to the origin of the mutation
Huntington disease (HD) is caused by an
expansion of a CAG repeat. This repeat is a dynamic
mutation that tends to undergo intergenerational instability.
We report the analysis of the CAG repeat in a large
population sample (2,000 chromosomes) covering all regions
of Portugal, and a haplotype study of (CAG)n and
(CCG)n repeats in 140 HD Portuguese families. Intermediate
class 2 alleles represented 3.0% of the population;
and two expanded alleles (36 and 40 repeats, 0.11%) were
found. There was no evidence for geographical clustering
of the intermediate or expanded alleles. The Portuguese
families showed three different HD founder haplotypes
associated with 7-, 9- or 10-CCG repeats, suggesting the
possibility of different origins for theHDmutation among
this population. The haplotype carrying the 7-CCG repeat
was the most frequent, both in normal and in expanded
alleles. In general, we propose that three mechanisms,
occurring at different times,may lead to the evolution from
normal CAGs to full expansion: first, a mutation bias towards
larger alleles; then, a stepwise process that could
explain the CAGdistributions observed in themore recent
haplotypes; and, finally, a pool of intermediate (class 2)
alleles more prone to give rise to expanded HD alleles.Fundação para a Ciência e a Tecnologia (FCT) - SFRH/BD/9759/
2003.Instituto de Genética Médica Jacinto Magalhães
Characterizing the gamma-ray long-term variability of PKS 2155-304 with H.E.S.S. and Fermi-LAT
Studying the temporal variability of BL Lac objects at the highest energies
provides unique insights into the extreme physical processes occurring in
relativistic jets and in the vicinity of super-massive black holes. To this
end, the long-term variability of the BL Lac object PKS 2155-304 is analyzed in
the high (HE, 100 MeV 200 GeV)
gamma-ray domain. Over the course of ~9 yr of H.E.S.S observations the VHE
light curve in the quiescent state is consistent with a log-normal behavior.
The VHE variability in this state is well described by flicker noise
(power-spectral-density index {\ss}_VHE = 1.10 +0.10 -0.13) on time scales
larger than one day. An analysis of 5.5 yr of HE Fermi LAT data gives
consistent results ({\ss}_HE = 1.20 +0.21 -0.23, on time scales larger than 10
days) compatible with the VHE findings. The HE and VHE power spectral densities
show a scale invariance across the probed time ranges. A direct linear
correlation between the VHE and HE fluxes could neither be excluded nor firmly
established. These long-term-variability properties are discussed and compared
to the red noise behavior ({\ss} ~ 2) seen on shorter time scales during
VHE-flaring states. The difference in power spectral noise behavior at VHE
energies during quiescent and flaring states provides evidence that these
states are influenced by different physical processes, while the compatibility
of the HE and VHE long-term results is suggestive of a common physical link as
it might be introduced by an underlying jet-disk connection.Comment: 11 pages, 16 figure
Detection of variable VHE gamma-ray emission from the extra-galactic gamma-ray binary LMC P3
Context. Recently, the high-energy (HE, 0.1-100 GeV) -ray emission
from the object LMC P3 in the Large Magellanic Cloud (LMC) has been discovered
to be modulated with a 10.3-day period, making it the first extra-galactic
-ray binary.
Aims. This work aims at the detection of very-high-energy (VHE, >100 GeV)
-ray emission and the search for modulation of the VHE signal with the
orbital period of the binary system.
Methods. LMC P3 has been observed with the High Energy Stereoscopic System
(H.E.S.S.); the acceptance-corrected exposure time is 100 h. The data set has
been folded with the known orbital period of the system in order to test for
variability of the emission. Energy spectra are obtained for the orbit-averaged
data set, and for the orbital phase bin around the VHE maximum.
Results. VHE -ray emission is detected with a statistical
significance of 6.4 . The data clearly show variability which is
phase-locked to the orbital period of the system. Periodicity cannot be deduced
from the H.E.S.S. data set alone. The orbit-averaged luminosity in the
TeV energy range is erg/s. A luminosity of erg/s is reached during 20% of the orbit. HE and VHE
-ray emissions are anti-correlated. LMC P3 is the most luminous
-ray binary known so far.Comment: 5 pages, 3 figures, 1 table, accepted for publication in A&
Detailed spectral and morphological analysis of the shell type SNR RCW 86
Aims: We aim for an understanding of the morphological and spectral
properties of the supernova remnant RCW~86 and for insights into the production
mechanism leading to the RCW~86 very high-energy gamma-ray emission. Methods:
We analyzed High Energy Spectroscopic System data that had increased
sensitivity compared to the observations presented in the RCW~86 H.E.S.S.
discovery publication. Studies of the morphological correlation between the
0.5-1~keV X-ray band, the 2-5~keV X-ray band, radio, and gamma-ray emissions
have been performed as well as broadband modeling of the spectral energy
distribution with two different emission models. Results:We present the first
conclusive evidence that the TeV gamma-ray emission region is shell-like based
on our morphological studies. The comparison with 2-5~keV X-ray data reveals a
correlation with the 0.4-50~TeV gamma-ray emission.The spectrum of RCW~86 is
best described by a power law with an exponential cutoff at TeV and a spectral index of ~. A static
leptonic one-zone model adequately describes the measured spectral energy
distribution of RCW~86, with the resultant total kinetic energy of the
electrons above 1 GeV being equivalent to 0.1\% of the initial kinetic
energy of a Type I a supernova explosion. When using a hadronic model, a
magnetic field of ~100G is needed to represent the measured data.
Although this is comparable to formerly published estimates, a standard
E spectrum for the proton distribution cannot describe the gamma-ray
data. Instead, a spectral index of ~1.7 would be required, which
implies that ~erg has been transferred into
high-energy protons with the effective density cm^-3. This
is about 10\% of the kinetic energy of a typical Type Ia supernova under the
assumption of a density of 1~cm^-3.Comment: accepted for publication by A&
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