A revised asteroid polarization-albedo relationship using WISE/NEOWISE data

We present a reanalysis of the relationship between asteroid albedo and polarization properties using the albedos derived from the Wide-field Infrared Survey Explorer. We find that the function that best describes this relation is a three-dimensional linear fit in the space of log(albedo)-log(polarization slope)-log(minimum polarization). When projected to two dimensions the parameters of the fit are consistent with those found in previous work. We also define p* as the quantity of maximal polarization variation when compared with albedo and present the best fitting albedo-p* relation. Some asteroid taxonomic types stand out in this three-dimensional space, notably the E, B, and M Tholen types, while others cluster in clumps coincident with the S- and C-complex bodies. We note that both low albedo and small (D<30 km) asteroids are under-represented in the polarimetric sample, and we encourage future polarimetric surveys to focus on these bodies.Comment: 16 pages, Accepted to Ap

The Circumstellar Disk of the Butterfly Star in Taurus

We present a model of the circumstellar environment of the so-called ``Butterfly Star'' in Taurus (IRAS 04302+2247). The appearance of this young stellar object is dominated by a large circumstellar disk seen edge-on and the light scattering lobes above the disk. The model is based on multi-wavelength continuum observations: Millimeter maps and high-resolution near-infrared images obtained with HST/NICMOS. It was found that the disk and envelope parameters are comparable with those of the circumstellar environment of other young stellar objects. A main result is that the dust properties must be different in the circumstellar disk and in the envelope: While a grain size distribution with grain radii up to 100 micron is required to reproduce the millimeter observations of the disk, the envelope is dominated by smaller grains similar to those of the interstellar medium. Preprint with high figure quality available at: http://spider.ipac.caltech.edu/staff/swolf/homepage/public/preprints/i04302.psComment: 32 pages, 9 figure

State-related electroencephalographic deviances in attention deficit hyperactivity disorder

The dynamic security region (DSR) of bulk power system has been accepted more and more in recent years for providing plenty of security information and good prospect in online application. This paper compares three linear approximations for the dynamic security region of network-reduction power systems. The three linear approximations are the Q-linear approximation based on the quadratic approximation of stability region, the L-linear approximation based on the linear approximation of stability region and the L0-linear approximation based on the invariant assumption of the normal vector for the boundary of the stability region corresponding to different control variable. The three linear approximations are all obtained with a same critical point lying just on the boundary of dynamic security region. The critical point is searched with numerical simulation. The accuracy of the three linear approximations is compared, using the linear approximation obtained with the curve fitting approach or the actual boundary of DSR searched as the benchmark. Simulation results in IEEE 3-machine 9-bus system and 10-machine 39-bus New England system show that all the three linear approximations display fairly accurate estimation. Furthermore, from the computational viewpoint, the L-linear and the L0-linear method are two alternative choices to approximate the dynamic security region.link_to_subscribed_fulltex

Expanding phenotype of hereditary fibrosing poikiloderma with tendon contractures, myopathy, and pulmonary fibrosis caused by FAM111B mutations: Report of an additional family raising the question of cancer predisposition and a short review of early-onset poikiloderma.

journal article2017 Mar2017 03 19importedHereditary fibrosing poikiloderma with tendon contractures, myopathy, and pulmonary fibrosis (POIKTMP [MIM#615704]) is an extremely rare syndromic form of autosomal dominant poikiloderma. This genetic disorder was first identified in a South African family in 2006.1 To date, 3 families and 9 independent sporadic cases have been reported.2-4 Here we report an additional family of POIKTMP and expand the clinical spectrum. We describe, for the first time to our knowledge, a pancreatic cancer in the clinical course in 1 patient

De novo intrachromosomal gene conversion from OPN1MW to OPN1LW in the male germline results in Blue Cone Monochromacy

X-linked cone dysfunction disorders such as Blue Cone Monochromacy and X-linked Cone Dystrophy are characterized by complete loss (of) or reduced L- and M- cone function due to defects in the OPN1LW/OPN1MW gene cluster. Here we investigated 24 affected males from 16 families with either a structurally intact gene cluster or at least one intact single (hybrid) gene but harbouring rare combinations of common SNPs in exon 3 in single or multiple OPN1LW and OPN1MW gene copies. We assessed twelve different OPN1LW/MW exon 3 haplotypes by semi-quantitative minigene splicing assay. Nine haplotypes resulted in aberrant splicing of ≥20% of transcripts including the known pathogenic haplotypes (i.e. ‘LIAVA’, ‘LVAVA’) with absent or minute amounts of correctly spliced transcripts, respectively. De novo formation of the ‘LIAVA’ haplotype derived from an ancestral less deleterious ‘LIAVS’ haplotype was observed in one family with strikingly different phenotypes among affected family members. We could establish intrachromosomal gene conversion in the male germline as underlying mechanism. Gene conversion in the OPN1LW/OPN1MW genes has been postulated, however, we are first to demonstrate a de novo gene conversion within the lineage of a pedigree

Energy Flow in the Hadronic Final State of Diffractive and Non-Diffractive Deep-Inelastic Scattering at HERA

An investigation of the hadronic final state in diffractive and non--diffractive deep--inelastic electron--proton scattering at HERA is presented, where diffractive data are selected experimentally by demanding a large gap in pseudo --rapidity around the proton remnant direction. The transverse energy flow in the hadronic final state is evaluated using a set of estimators which quantify topological properties. Using available Monte Carlo QCD calculations, it is demonstrated that the final state in diffractive DIS exhibits the features expected if the interaction is interpreted as the scattering of an electron off a current quark with associated effects of perturbative QCD. A model in which deep--inelastic diffraction is taken to be the exchange of a pomeron with partonic structure is found to reproduce the measurements well. Models for deep--inelastic $ep$ scattering, in which a sizeable diffractive contribution is present because of non--perturbative effects in the production of the hadronic final state, reproduce the general tendencies of the data but in all give a worse description.Comment: 22 pages, latex, 6 Figures appended as uuencoded fil

A Search for Selectrons and Squarks at HERA

Data from electron-proton collisions at a center-of-mass energy of 300 GeV are used for a search for selectrons and squarks within the framework of the minimal supersymmetric model. The decays of selectrons and squarks into the lightest supersymmetric particle lead to final states with an electron and hadrons accompanied by large missing energy and transverse momentum. No signal is found and new bounds on the existence of these particles are derived. At 95% confidence level the excluded region extends to 65 GeV for selectron and squark masses, and to 40 GeV for the mass of the lightest supersymmetric particle.Comment: 13 pages, latex, 6 Figure

A possible cranio-oro-facial phenotype in Cockayne syndrome

BACKGROUND: Cockayne Syndrome CS (Type A - CSA; or CS Type I OMIM #216400) (Type B - CSB; or CS Type II OMIM #133540) is a rare autosomal recessive neurological disease caused by defects in DNA repair characterized by progressive cachectic dwarfism, progressive intellectual disability with cerebral leukodystrophy, microcephaly, progressive pigmentary retinopathy, sensorineural deafness photosensitivity and possibly orofacial and dental anomalies. METHODS: We studied the cranio-oro-facial status of a group of 17 CS patients from 15 families participating in the National Hospital Program for Clinical Research (PHRC) 2005 >. All patients were examined by two investigators using the Diagnosing Dental Defects Database (D[4]/phenodent) record form. RESULTS: Various oro-facial and dental anomalies were found: retrognathia; micrognathia; high- arched narrow palate; tooth crowding; hypodontia (missing permanent lateral incisor, second premolars or molars), screwdriver shaped incisors, microdontia, radiculomegaly, and enamel hypoplasia. Eruption was usually normal. Dental caries was associated with enamel defects, a high sugar/carbohydrate soft food diet, poor oral hygiene and dry mouth. Cephalometric analysis revealed mid-face hypoplasia, a small retroposed mandible and hypo-development of the skull. CONCLUSION: CS patients may have associated oro-dental features, some of which may be more frequent in CS children - some of them being described for the first time in this paper (agenesis of second permanent molars and radiculomegaly). The high susceptibility to rampant caries is related to a combination of factors as well as enamel developmental defects. Specific attention to these anomalies may contribute to diagnosis and help plan management

Mutations in the latent TGF-beta binding protein 3 (LTBP3) gene cause brachyolmia with amelogenesis imperfecta

Inherited dental malformations constitute a clinically and genetically heterogeneous group of disorders. Here, we report on four families, three of them consanguineous, with an identical phenotype, characterized by significant short stature with brachyolmia and hypoplastic amelogenesis imperfecta (AI) with almost absent enamel. This phenotype was first described in 1996 by Verloes et al. as an autosomal recessive form of brachyolmia associated with AI. Whole-exome sequencing resulted in the identification of recessive hypomorphic mutations including deletion, nonsense and splice mutations, in the LTBP3 gene, which is involved in the TGF-beta signaling pathway. We further investigated gene expression during mouse development and tooth formation. Differentiated ameloblasts synthesizing enamel matrix proteins and odontoblasts expressed the gene. Study of an available knockout mouse model showed that the mutant mice displayed very thin to absent enamel in both incisors and molars, hereby recapitulating the AI phenotype in the human disorder