Some considerations of manned extravehicular activities in assembly and operation of large space structures

Concepts and considerations for manned extravehicular activities in assembly and operation of large space structure

A semi-atomistic model for flaw-induced fracture in non-ductile materials

Semiatomistic model for nonductile fracture based on analysis of static and dynamic stress concentrations in uniaxial fiber-matrix composite material

Thermally stable macro-composite structures

Studies and experiments on the use of bimetallic lattice structures for determining and controlling thermal expansion characteristics are discussed

Pressurized structures of high mobility

Filamentary fabric made with slip-net principle principle for pressurized suit

Large low-frequency orbiting radio telescope

Feasibility of orbiting paraboloidal antenna for low frequency radio astronom

A contribution to the micromechanics of composite materials - Stresses and failure mechanisms induced by inclusions

Stress field concentrations produced by cylindrical inclusions in composite material

Compressive strength of boron-metal composites

Compressive strength and failure modes of boron fiber-magnesium matrix composit

Axisymmetric and cylindrical isostabiloids

Differential equations for compression loaded axisymmetric cylindrical structure

Heliogyro solar sailer Summary report

Large solar sail vehicle to operate in manner of helicopter roto

Electroretinography Reveals Difference in Cone Function between Syndromic and Nonsyndromic USH2A Patients

Usher syndrome is an inherited and irreversible disease that manifests as retinitis pigmentosa (RP) and bilateral neurosensory hearing loss. Mutations in Usherin 2A (USH2A) are not only a frequent cause of Usher syndrome, but also nonsyndromic RP. Although gene-and cell-based therapies are on the horizon for RP and Usher syndrome, studies characterizing natural disease are lacking. In this retrospective analysis, retinal function of USH2A patients was quantified with electroretinography. Both groups had markedly reduced rod and cone responses, but nonsyndromic USH2A patients had 30 Hz-flicker electroretinogram amplitudes that were significantly higher than syndromic patients, suggesting superior residual cone function. There was a tendency for Usher syndrome patients to have a higher distribution of severe mutations, and alleles in this group had a higher odds of containing nonsense or frame-shift mutations. These data suggest that the previously reported severe visual phenotype seen in syndromic USH2A patients could relate to a greater extent of cone dysfunction. Additionally, a genetic threshold may exist where mutation burden relates to visual phenotype and the presence of hearing deficits. The auditory phenotype and allelic hierarchy observed among patients should be considered in prospective studies of disease progression and during enrollment for future clinical trials.National Institute of HealthNational Cancer InstituteResearch to Prevent Blindness (RPB)RPB, New York, NY, USARPBInternational Council of Ophthalmology - Retina Research FoundationNIHTistou and Charlotte Kerstan FoundationSchneeweiss Stem Cell Fund, New York StateFoundation Fighting Blindness New York Regional Research CenterCrowley Family FundGebroe Family FoundationColumbia Univ, Dept Ophthalmol, Med Ctr, Jonas Childrens Vis Care, New York, NY 10027 USAColumbia Univ, Dept Ophthalmol, Med Ctr, Bernard & Shirlee Brown Glaucoma Lab, New York, NY 10027 USANew York Presbyterian Hosp, Edward S Harkness Eye Inst, New York, NY 10034 USASuny Downstate Med Ctr, Brooklyn, NY 11203 USAUniv Fed Espirito Santo, Dept Ophthalmol, Vitoria, BrazilUniv Fed Sao Paulo, Dept Ophthalmol, Sao Paulo, BrazilColumbia Univ, Dept Biostat, New York, NY USAUniv Montreal, Dept Ophthalmol, Montreal, PQ, CanadaShanghai Jiao Tong Univ, Sch Med, Xin Hua Hosp, Dept Ophthalmol, Shanghai, Peoples R ChinaColumbia Univ, Inst Human Nutr, Dept Pathol & Cell Biol, Stem Cell Initiat CSCI,Coll Phys & Surg, New York, NY 10032 USAStanford Univ, Dept Ophthalmol, Om Lab, Byers Eye Inst, Palo Alto, CA 94304 USAUniv Fed Sao Paulo, Dept Ophthalmol, Sao Paulo, BrazilWeb of Scienc