Progressive myoclonus epilepsy KCNC1 variant causes a developmental dendritopathy

OBJECTIVE: Mutations in KCNC1 can cause severe neurological dysfunction, including intellectual disability, epilepsy, and ataxia. The Arg320His variant, which occurs in the voltage-sensing domain of the channel, causes a highly penetrant and specific form of progressive myoclonus epilepsy with severe ataxia, designated myoclonus epilepsy and ataxia due to potassium channel mutation (MEAK). KCNC1 encodes the voltage-gated potassium channel KV 3.1, a channel that is important for enabling high-frequency firing in interneurons, raising the possibility that MEAK is associated with reduced interneuronal function. METHODS: To determine how this variant triggers MEAK, we expressed KV 3.1bR320H in cortical interneurons in vitro and investigated the effects on neuronal function and morphology. We also performed electrophysiological recordings of oocytes expressing KV 3.1b to determine whether the mutation introduces gating pore currents. RESULTS: Expression of the KV 3.1bR320H variant profoundly reduced excitability of mature cortical interneurons, and cells expressing these channels were unable to support high-frequency firing. The mutant channel also had an unexpected effect on morphology, severely impairing neurite development and interneuron viability, an effect that could not be rescued by blocking KV 3 channels. Oocyte recordings confirmed that in the adult KV 3.1b isoform, R320H confers a dominant negative loss-of-function effect by slowing channel activation, but does not introduce potentially toxic gating pore currents. SIGNIFICANCE: Overall, our data suggest that, in addition to the regulation of high-frequency firing, KV 3.1 channels play a hitherto unrecognized role in neuronal development. MEAK may be described as a developmental dendritopathy

Field-induced coupled superconductivity and spin density wave order in the Heavy Fermion compound CeCoIn5

The high field superconducting state in CeCoIn5 has been studied by transverse field muon spin rotation measurements with an applied field parallel to the crystallographic c-axis close to the upper critical field Hc2 = 4.97 T. At magnetic fields >= 4.8 T the muon Knight shift is enhanced and the superconducting transition changes from second order towards first order as predicted for Pauli-limited superconductors. The field and temperature dependence of the transverse muon spin relaxation rate sigma reveal paramagnetic spin fluctuations in the field regime from 2 T < H < 4.8 T. In the normal state close to Hc2 correlated spin fluctuations as described by the self consistent renormalization theory are observed. The results support the formation of a mode-coupled superconducting and antiferromagnetically ordered phase in CeCoIn5 for H directed parallel to the c-axis.Comment: 5 paes, 4 figure

Imaging Three Dimensional Two-particle Correlations for Heavy-Ion Reaction Studies

We report an extension of the source imaging method for analyzing three-dimensional sources from three-dimensional correlations. Our technique consists of expanding the correlation data and the underlying source function in spherical harmonics and inverting the resulting system of one-dimensional integral equations. With this strategy, we can image the source function quickly, even with the finely binned data sets common in three-dimensional analyses.Comment: 13 pages, 11 figures, submitted to Physical Review

Pd/Cu Site Interchange and Non-Fermi-Liquid Behavior in UCu_4Pd

X-ray-absorption fine-structure measurements of the local structure in UCu_4Pd are described which indicate a probable lattice-disorder origin for non-Fermi-liquid behavior in this material. Short Pd-Cu distances are observed, consistent with 24 +/- 3% of the Pd atoms occupying nominally Cu sites. A "Kondo disorder" model, based on the effect on the local Kondo temperature T_K of this interchange and some additional bond-length disorder, agrees quantitatively with previous experimental susceptibility data, and therefore also with specific heat and magnetic resonance experiments.Comment: 4 pages, 3 PostScript figures, to be published in PR

Identification of Non-unitary triplet pairing in a heavy Fermion superconductor UPt_3

A NMR experiment recently done by Tou et al. on a heavy Fermion superconductor UPt$_3$ is interpreted in terms of a non-unitary spin-triplet pairing state which we have been advocating. The proposed state successfully explains various aspects of the seemingly complicated Knight shift behaviors probed for major orientations, including a remarkable d-vector rotation under weak fields. This entitles UPt$_3$ as the first example that a charged many body system forms a spin-triplet odd-par ity pairing at low temperatures and demonstrates unambiguously that the putative spin-orbit coupling in UPt$_3$ is weak.Comment: 4 pages, 2 eps figures, to be published in J. Phys. Soc. Jpn. 67 (1998) No.

Observation of two time scales in the ferromagnetic manganite La(1-x)Ca(x)MnO(3), x = 0.3

We report new zero-field muon spin relaxation and neutron spin echo measurements in ferromagnetic (FM) (La,Ca)MnO3 which taken together suggest two spatially separated regions in close proximity possessing very different Mn-ion spin dynamics. One region corresponds to an extended cluster which displays 'critical slowing down' near Tc and an increasing volume fraction below Tc. The second region possesses more slowly fluctuating spins and a decreasing volume fraction below Tc. These data are discussed in terms of the growth of small polarons into overlapping regions of correlated spins below Tc, resulting in a microscopically inhomogeneous FM transition.Comment: 10 pages, 3 figure

Muon Spin Relaxation Study of (La, Ca)MnO3

We report predominantly zero field muon spin relaxation measurements in a series of Ca-doped LaMnO_3 compounds which includes the colossal magnetoresistive manganites. Our principal result is a systematic study of the spin-lattice relaxation rates 1/T_1 and magnetic order parameters in the series La_{1-x}Ca_xMnO_3, x = 0.0, 0.06, 0.18, 0.33, 0.67 and 1.0. In LaMnO_3 and CaMnO_3 we find very narrow critical regions near the Neel temperatures T_N and temperature independent 1/T_1 values above T_N. From the 1/T_1 in LaMnO_3 we derive an exchange integral J = 0.83 meV which is consistent with the mean field expression for T_N. All of the doped manganites except CaMnO_3 display anomalously slow, spatially inhomogeneous spin-lattice relaxation below their ordering temperatures. In the ferromagnetic (FM) insulating La_{0.82}Ca_{0.18}MnO_3 and ferromagnetic conducting La_{0.67}Ca_{0.33}MnO_3 systems we show that there exists a bi-modal distribution of \muSR rates \lambda_f and \lambda_s associated with relatively 'fast' and 'slow' Mn fluctuation rates, respectively. A physical picture is hypothesized for these FM phases in which the fast Mn rates are due to overdamped spin waves characteristic of a disordered FM, and the slower Mn relaxation rates derive from distinct, relatively insulating regions in the sample. Finally, likely muon sites are identified, and evidence for muon diffusion in these materials is discussed.Comment: 21 pages, 17 figure

Evidence for a two component magnetic response in UPt3

The magnetic response of the heavy fermion superconductor UPt_3 has been investigated on a microscopic scale by muon Knight shift studies. Two distinct and isotropic Knight shifts have been found for the field in the basal plane. While the volume fractions associated with the two Knight shifts are approximately equal at low and high temperatures, they show a dramatic and opposite temperature dependence around T_N. Our results are independent on the precise muon localization site. We conclude that UPt_3 is characterized by a two component magnetic response.Comment: 5 pages, 4 figure