Functional Characterisation of Alpha-Galactosidase A Mutations as a Basis for a New Classification System in Fabry Disease

This is an open-access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.The study has been supported partially by an unrestricted scientific grant from Shire Human Genetic Therapies (Germany

Activation measurement of the 3He(alpha,gamma)7Be cross section at low energy

The nuclear physics input from the 3He(alpha,gamma)7Be cross section is a major uncertainty in the fluxes of 7Be and 8B neutrinos from the Sun predicted by solar models and in the 7Li abundance obtained in big-bang nucleosynthesis calculations. The present work reports on a new precision experiment using the activation technique at energies directly relevant to big-bang nucleosynthesis. Previously such low energies had been reached experimentally only by the prompt-gamma technique and with inferior precision. Using a windowless gas target, high beam intensity and low background gamma-counting facilities, the 3He(alpha,gamma)7Be cross section has been determined at 127, 148 and 169 keV center-of-mass energy with a total uncertainty of 4%. The sources of systematic uncertainty are discussed in detail. The present data can be used in big-bang nucleosynthesis calculations and to constrain the extrapolation of the 3He(alpha,gamma)7Be astrophysical S-factor to solar energies

Luttinger-Liquid Behavior in the Alternating Spin-Chain System Copper Nitrate

We determine the phase diagram of copper nitrate Cu(NO$_3$)$_2\cdot$2.5D$_2$O in the context of quantum phase transitions and novel states of matter. We establish this compound as an ideal candidate to study quasi-1D Luttinger liquids, 3D Bose-Einstein-Condensation of triplons, and the crossover between 1D and 3D physics. Magnetocaloric effect, magnetization, and neutron scattering data provide clear evidence for transitions into a Luttinger liquid regime and a 3D long-range ordered phase as function of field and temperature. Theoretical simulations of this model material allow us to fully establish the phase diagram and to discuss it in the context of dimerized spin systems.Comment: 5 pages, 4 figure

Thermal neutron induced (n,p) and (n,alpha) reactions on 37Ar

The 37Ar(n_th,alpha)34S and 37Ar(n_th,p)37Cl reactions were studied at the high flux reactor of the ILL in Grenoble. For the 37Ar(n_th,alpha_0) and 37Ar(n_th,p) reaction cross sections, values of (1070+/-80)b and (37+/-4)b, respectively, were obtained. Both values are about a factor 2 smaller than results of older measurements. The observed suppression of the 37(n_th,alpha_1) transition could be verified from theoretical considerations. Finally, evidence was found for the two-step 37Ar(n_th,gamma-alpha) process.Comment: 11 pages, 5 figures, accepted for publication in Nuclear Physics

Thermonuclear Reaction Rate of 23Mg(p,gamma)24$Al

Updated stellar rates for the reaction 23Mg(p,gamma)24Al are calculated by using all available experimental information on 24Al excitation energies. Proton and gamma-ray partial widths for astrophysically important resonances are derived from shell model calculations. Correspondences of experimentally observed 24Al levels with shell model states are based on application of the isobaric multiplet mass equation. Our new rates suggest that the 23Mg(p,gamma)24Al reaction influences the nucleosynthesis in the mass A>20 region during thermonuclear runaways on massive white dwarfs.Comment: 13 pages (uses Revtex) including 3 postscript figures (uses epsfig.sty), accepted for publication in Phys. Rev.

Impact of a revised 25^{25}Mg(p,γ\gamma)26^{26}Al reaction rate on the operation of the Mg-Al cycle

Proton captures on Mg isotopes play an important role in the Mg-Al cycle active in stellar H-burning regions. In particular, low-energy nuclear resonances in the $^{25}$Mg(p,$\gamma$)$^{26}$Al reaction affect the production of radioactive $^{26}$Al$^{gs}$ as well as the resulting Mg/Al abundance ratio. Reliable estimations of these quantities require precise measurements of the strengths of low-energy resonances. Based on a new experimental study performed at LUNA, we provide revised rates of the $^{25}$Mg(p,$\gamma$)$^{26}$Al$^{gs}$ and the $^{25}$Mg(p,$\gamma$)$^{26}$Al$^{m}$ reactions with corresponding uncertainties. In the temperature range 50 to 150 MK, the new recommended rate of the $^{26}$Al$^{m}$ production is up to 5 times higher than previously assumed. In addition, at T$=100$ MK, the revised total reaction rate is a factor of 2 higher. Note that this is the range of temperature at which the Mg-Al cycle operates in an H-burning zone. The effects of this revision are discussed. Due to the significantly larger $^{25}$Mg(p,$\gamma$)$^{26}$Al$^{m}$ rate, the estimated production of $^{26}$Al$^{gs}$ in H-burning regions is less efficient than previously obtained. As a result, the new rates should imply a smaller contribution from Wolf-Rayet stars to the galactic $^{26}$Al budget. Similarly, we show that the AGB extra-mixing scenario does not appear able to explain the most extreme values of $^{26}$Al/$^{27}$Al, i.e. $>10^{-2}$, found in some O-rich presolar grains. Finally, the substantial increase of the total reaction rate makes the hypothesis of a self-pollution by massive AGBs a more robust explanation for the Mg-Al anticorrelation observed in Globular-Cluster stars

The Bare Astrophysical S(E) Factor of the 7Li(p, α)α Reaction

The astrophysically important 7Li(p, α)α reaction has been studied via the Trojan horse method in the energy range E = 10-400 keV. A new theoretical description, based on the distorted-wave Born approximation approach, allows one to extract information on the bare astrophysical S-factor, Sb(E), with Sb(0) = 55 ± 3 keV barns. The results are compared with direct experimental data leading to a model-independent value of the electron screening potential energy, Ue = 330 ± 40 eV, much higher than the adiabatic limit Uad = 175 eV

Ba3 Mx Ti3−x O9 (M = Ir, Rh): A family of 5d/4d-based diluted quantum spin liquids

We report the structural and magnetic properties of the 4d (M = Rh) based and 5d (M = Ir) based systems Ba3Mx Ti3−x O9 (nominally x = 0.5, 1). The studied compositions were found to crystallize in a hexagonal structure with the centrosymmetric space group P 63/mmc. The structures comprise of A2O9 polyhedra [with the A site (possibly) statistically occupied by M and Ti] in which pairs of transition metal ions are stacked along the crystallographic c axis. These pairs form triangular bilayers in the ab plane. The magnetic Rh and Ir ions occupy these bilayers, diluted by Ti ions even for x = 1. These bilayers are separated by a triangular layer which is dominantly occupied by Ti ions. From magnetization measurements we infer strong antiferromagnetic couplings for all of the materials but the absence of any spin-freezing or spin-ordering down to 2 K. Further, specific heat measurements down to 0.35 K show no sign of a phase transition for any of the compounds. Based on these thermodynamic measurements we propose the emergence of a quantum spin liquid ground state for Ba3Rh0.5Ti2.5O9, and Ba3Ir0.5Ti2.5O9, in addition to the already reported Ba3IrTi2O9

Nuclear masses set bounds on quantum chaos

It has been suggested that chaotic motion inside the nucleus may significantly limit the accuracy with which nuclear masses can be calculated. Using a power spectrum analysis we show that the inclusion of additional physical contributions in mass calculations, through many-body interactions or local information, removes the chaotic signal in the discrepancies between calculated and measured masses. Furthermore, a systematic application of global mass formulas and of a set of relationships among neighboring nuclei to more than 2000 nuclear masses allows to set an unambiguous upper bound for the average errors in calculated masses which turn out to be almost an order of magnitude smaller than estimated chaotic components.Comment: 4 pages, Accepted for publication in Physical Review Letter