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Mutations in ATP8B1 can lead to familial intrahepatic cholestasis type 1 (FIC1) deficiency, or progressive familial intrahepatic cholestasis type 1 (PFIC1). The rarity of FIC1 deficiency has largely prevented a detailed analysis of its natural history, effects of predicted protein truncating mutations (PPTMs), and possible associations of serum bile acid (sBA) concentrations and surgical biliary diversion (SBD) with long-term outcome. We aimed to provide novel insights by using the largest genetically defined cohort of FIC1 deficiency patients to date. This multicenter, combined retrospective and prospective study included 130 patients with compound heterozygous or homozygous predicted pathogenic ATP8B1 variants. Patients were categorized according to the number of PPTMs (i.e., splice site, frameshift due to deletion or insertion, nonsense, duplication); FIC1-A (n=67; no PPTM), FIC1-B (n=29; one PPTM) or FIC1-C (n=34; two PPTMs). Survival analysis showed an overall native liver survival (NLS) of 44% at age 18y. NLS was comparable between FIC1-A, FIC1-B, and FIC1-C (%NLS at age 10y: 67%, 41%, and 59%, respectively; P=0.12), despite FIC1-C undergoing SBD less often (%SBD at age 10y: 65%, 57%, and 45%, respectively; P=0.03). sBAs at presentation were negatively associated with NLS (NLS at age 10y; sBAs <194 µmol/L: 49% versus sBAs ≥194 µmol/L: 15%; P=0.03). SBD decreased sBAs (230 [125-282] to 74 [11-177] μmol/L; P=0.005). SBD (HR 0.55, 95% CI 0.28-1.03, P=0.06) and post-SBD sBA concentrations <65μmol/L (P=0.05) tended to be associated with improved NLS. Conclusion: Less than half of FIC1 deficiency patients reach adulthood with native liver. The number of PPTMs did not associate with the natural history or prognosis of FIC1 deficiency. sBA concentrations at initial presentation and after SBD provide limited prognostic information on long-term NLS.

Catalytic hydropyrolysis combines fast pyrolysis with catalytic upgrading by hydrodeoxygenation (HDO) in a single step for the production of fuel oil from biomass in which reactive pyrolysis vapors are upgraded before condensation. Catalyst activity and lifetime is challenged by carbon deposition and by the presence of alkali, nitrogen, sulfur and water. In this contribution, a combined experimental, characterization (in-situ Extended X-Ray Absorption Fine Structure, EXAFS) and theoretical (Density Functional Theory, DFT) study of catalytic HDO of biomass model compounds over MoS2 based catalysts is presented with emphasis on the influence of water and H2S.

ARKIVOC: vol. 2010, no. 8, (issn) 1551-7012, (dlps) 5550190.0011.806, This work is licensed under a Creative Commons Attribution-NonCommercial 3.0 License. Please contact mpub-help@umich.edu to use this work in a way not covered by the license.

ARKIVOC: vol. 2010, no. 8, (issn) 1551-7012, (dlps) 5550190.0011.806, This work is licensed under a Creative Commons Attribution-NonCommercial 3.0 License. Please contact mpub-help@umich.edu to use this work in a way not covered by the license.

We present a modified beat-frequency modulated accretion model appropriate to a description of the horizontal branch oscillations of Z-type low-mass x-ray binaries. This model incorporates several new physical elements: (1) magnetic screening by the accretion disk and neutron star, (2) radiation pressure dominance in the inner disk, and (3) stellar rotation effects. Fitted to observational data, the model constrains the surface magnetic field in a typical LMXB Z source to be less than 10(exp 9) G and the stellar spin frequency to be greater than 400 Hz. The predicted high stellar spin frequency falls in a range where observational upper limits on pulsation are not a constraint. The derived magnetic field and spin frequency range are compatible with the fastest radio pulsars.

Recently a new technique for characterizing the noise processes affecting oscillators was introduced [1] [2]. This technique minimizes the difference between the estimates of several different variances and their values as predicted by the standard power law model of noise. The method outlined in this paper makes two significant advancements: it uses exclusively time domain variances so that deterministic parameters such as linear frequency drift may be estimated, and it correctly fits the estimates using the chi-square distribution. These changes permit a more accurate fitting at long time intervals where there is the least information. This technique has been applied to both simulated and real data with excellent results., See also ADA267301. Presented at the Annual Precise Time and Time Interval (PTTI) Applications and Planning Meeting (24th), McLean, VA, 1-3 Dec 1992.

Pteridophytes, http://name.umdl.umich.edu/IC-HERB00IC-X-1601279%5DMICH-V-1601279, https://quod.lib.umich.edu/cgi/i/image/api/thumb/herb00ic/1601279/MICH-V-1601279/!250,250, The University of Michigan Library provides access to these materials for educational and research purposes. Some materials may be protected by copyright. If you decide to use any of these materials, you are responsible for making your own legal assessment and securing any necessary permission. If you have questions about the collection, please contact the Herbarium professional staff: herb-dlps-help@umich.edu. If you have concerns about the inclusion of an item in this collection, please contact Library Information Technology: libraryit-info@umich.edu., https://www.lib.umich.edu/about-us/policies/copyright-policy

Pteridophytes, http://name.umdl.umich.edu/IC-HERB00IC-X-1600610%5DMICH-V-1600610, https://quod.lib.umich.edu/cgi/i/image/api/thumb/herb00ic/1600610/MICH-V-1600610/!250,250, The University of Michigan Library provides access to these materials for educational and research purposes. Some materials may be protected by copyright. If you decide to use any of these materials, you are responsible for making your own legal assessment and securing any necessary permission. If you have questions about the collection, please contact the Herbarium professional staff: herb-dlps-help@umich.edu. If you have concerns about the inclusion of an item in this collection, please contact Library Information Technology: libraryit-info@umich.edu., https://www.lib.umich.edu/about-us/policies/copyright-policy

Angiosperms, http://name.umdl.umich.edu/IC-HERB00IC-X-1666231%5DMICH-V-1666231, https://quod.lib.umich.edu/cgi/i/image/api/thumb/herb00ic/1666231/MICH-V-1666231/!250,250, The University of Michigan Library provides access to these materials for educational and research purposes. Some materials may be protected by copyright. If you decide to use any of these materials, you are responsible for making your own legal assessment and securing any necessary permission. If you have questions about the collection, please contact the Herbarium professional staff: herb-dlps-help@umich.edu. If you have concerns about the inclusion of an item in this collection, please contact Library Information Technology: libraryit-info@umich.edu., https://www.lib.umich.edu/about-us/policies/copyright-policy

Pteridophytes, http://name.umdl.umich.edu/IC-HERB00IC-X-1601279%5DMICH-V-1601279, https://quod.lib.umich.edu/cgi/i/image/api/thumb/herb00ic/1601279/MICH-V-1601279/!250,250, The University of Michigan Library provides access to these materials for educational and research purposes. Some materials may be protected by copyright. If you decide to use any of these materials, you are responsible for making your own legal assessment and securing any necessary permission. If you have questions about the collection, please contact the Herbarium professional staff: herb-dlps-help@umich.edu. If you have concerns about the inclusion of an item in this collection, please contact Library Information Technology: libraryit-info@umich.edu., https://www.lib.umich.edu/about-us/policies/copyright-policy

Pteridophytes, http://name.umdl.umich.edu/IC-HERB00IC-X-1600610%5DMICH-V-1600610, https://quod.lib.umich.edu/cgi/i/image/api/thumb/herb00ic/1600610/MICH-V-1600610/!250,250, The University of Michigan Library provides access to these materials for educational and research purposes. Some materials may be protected by copyright. If you decide to use any of these materials, you are responsible for making your own legal assessment and securing any necessary permission. If you have questions about the collection, please contact the Herbarium professional staff: herb-dlps-help@umich.edu. If you have concerns about the inclusion of an item in this collection, please contact Library Information Technology: libraryit-info@umich.edu., https://www.lib.umich.edu/about-us/policies/copyright-policy

Angiosperms, http://name.umdl.umich.edu/IC-HERB00IC-X-1666231%5DMICH-V-1666231, https://quod.lib.umich.edu/cgi/i/image/api/thumb/herb00ic/1666231/MICH-V-1666231/!250,250, The University of Michigan Library provides access to these materials for educational and research purposes. Some materials may be protected by copyright. If you decide to use any of these materials, you are responsible for making your own legal assessment and securing any necessary permission. If you have questions about the collection, please contact the Herbarium professional staff: herb-dlps-help@umich.edu. If you have concerns about the inclusion of an item in this collection, please contact Library Information Technology: libraryit-info@umich.edu., https://www.lib.umich.edu/about-us/policies/copyright-policy

Fungi, http://name.umdl.umich.edu/IC-HERB00IC-X-271198%5DMICH-F-271198, https://quod.lib.umich.edu/cgi/i/image/api/thumb/herb00ic/271198/MICH-F-271198/!250,250, The University of Michigan Library provides access to these materials for educational and research purposes. Some materials may be protected by copyright. If you decide to use any of these materials, you are responsible for making your own legal assessment and securing any necessary permission. If you have questions about the collection, please contact the Herbarium professional staff: herb-dlps-help@umich.edu. If you have concerns about the inclusion of an item in this collection, please contact Library Information Technology: libraryit-info@umich.edu., https://www.lib.umich.edu/about-us/policies/copyright-policy

Angiosperms, http://name.umdl.umich.edu/IC-HERB00IC-X-1439633%5DMICH-V-1439633, https://quod.lib.umich.edu/cgi/i/image/api/thumb/herb00ic/1439633/MICH-V-1439633/!250,250, The University of Michigan Library provides access to these materials for educational and research purposes. Some materials may be protected by copyright. If you decide to use any of these materials, you are responsible for making your own legal assessment and securing any necessary permission. If you have questions about the collection, please contact the Herbarium professional staff: herb-dlps-help@umich.edu. If you have concerns about the inclusion of an item in this collection, please contact Library Information Technology: libraryit-info@umich.edu., https://www.lib.umich.edu/about-us/policies/copyright-policy

Angiosperms, http://name.umdl.umich.edu/IC-HERB00IC-X-1439633%5DMICH-V-1439633, https://quod.lib.umich.edu/cgi/i/image/api/thumb/herb00ic/1439633/MICH-V-1439633/!250,250, The University of Michigan Library provides access to these materials for educational and research purposes. Some materials may be protected by copyright. If you decide to use any of these materials, you are responsible for making your own legal assessment and securing any necessary permission. If you have questions about the collection, please contact the Herbarium professional staff: herb-dlps-help@umich.edu. If you have concerns about the inclusion of an item in this collection, please contact Library Information Technology: libraryit-info@umich.edu., https://www.lib.umich.edu/about-us/policies/copyright-policy

Fungi, http://name.umdl.umich.edu/IC-HERB00IC-X-280658%5DMICH-F-280658, https://quod.lib.umich.edu/cgi/i/image/api/thumb/herb00ic/280658/MICH-F-280658/!250,250, The University of Michigan Library provides access to these materials for educational and research purposes. Some materials may be protected by copyright. If you decide to use any of these materials, you are responsible for making your own legal assessment and securing any necessary permission. If you have questions about the collection, please contact the Herbarium professional staff: herb-dlps-help@umich.edu. If you have concerns about the inclusion of an item in this collection, please contact Library Information Technology: libraryit-info@umich.edu., https://www.lib.umich.edu/about-us/policies/copyright-policy

An electrooptic (EO) sampling system suitable for high speed measurements on gallium arsenide (GaAs) integrated circuits (IC's) was developed. This measurement technique is based on the linear electrooptic effect in GaAs. Using a longitudinal probing geometry, sub bandgap energy infrared light is passed through the substrate of a GaAs IC, reflected off some circuit metallization, and passed through a polarizer, resulting in an intensity change of the light past the polarizer proportional to the voltage across the substrate.

Four methods for growing thin films of high Tc superconductors have been developed and properties of the films have been investigated. A new superconducting structure of composition Y2Ba4Cu8O20- has been discovered. In a number of ways it has properties which are better than those of the YBa2Cu3O7 structures. It is less anisotrophic in the CuO2 (conducting planes) and therefore has less (or no) twinning. It retains oxygen under vacuum conditions and thus may be superior for making planar Josephson Junctions. Keywords: Yttrium compounds, Barium, Copper compounds, Oxides, Epitaxial growth, Vapor deposition.

A long pulse, high brightness 45 MeV linear accelerator has been constructed for use as a driver for free electron laser and beam wave experiments, and as the first section of a 1 GeV injector for a high brightness electron storage ring. This contract has led to: 1. demonstration of the highest gradient and longest pulse length yet achieved in a 3 meter S-band SLAC-type travelling wave accelerator section; and 2. demonstration of the high current and high current density attainable through the use of a strong microwave field to replace the dc electric field usually used in linac electron guns.

In the first part of this research, specially designed hapten-phospholipid molecules were synthesized to enable the binding of immunoglobulins to artificial phospholipid membranes. In subsequent work, using these immunoglobulin-membrane complexes, electron microscopy was used to reveal the detailed crystalline structure of a two dimensional (2D) immunoglobulin crystal attached to an electron microscope grid. These studies enabled the discovery of a new conformational arrangement of crystals formed from intact immunoglobulin molecules. In a third part of the study, monolayers of protein-membrane complex (in this case the purple membrane from halobacterium halobium) were deposited on a single crystal silicon substrate. Using the technique of grazing incidence X-ray diffraction, diffraction patterns from a single monolayer of protein-lipid complex were obtained for the first time.

This research is concerned with methods for achieving temperature compensation of surface-type acoustic wave resonators and delay lines in high coupling materials such as lithium niobate and lithium tantalate. Emphasis in placed on surface transverse waves that is horizontally polarized shear waves trapped on the surface by a grating structure consisting of grooves, mass loading strips, conducting strips, or combinations thereof.

The purpose of the work was to study the low rf-field loss of A15 superconducting compounds. This type of superconducting material has potential for application to the construction of microwave energy storage cavities whose energy can be dumped in a very short time so as to produce a very large microwave power pulse. An apparatus, using a calorimetric technique, was developed to measure the surface resistance of small-area superconducting samples deposited on sapphire substrates. Both a Nb and a Nb3Sn sample (the latter in the A15 structure) were prepared, characterized and measured. These initial measurements revealed additional losses that are most likely due to the region near the superconductor-sapphire interface. Measurements of the superconducting transition temperature were made as a function of film thickness to study the effect of the interface. The interface losses can be avoided in practice, and in our measurements they can be circumvented by employing a circularly cylindrical geometry which shields the interface region from the rf fields. In the course of developing the surface resistance apparatus, dielectric loss measurements were made on pure sapphire samples.

The objective of this program is the determination of the characteristics and capabilities of laser amplifiers and oscillators based on the stimulated emission of radiation by free electrons in a spatially periodic magnetic field. The previous effort in this program was directed towards the measurement of the single pass gain and the dependence of the gain on electron energy and current, the electron beam radius and divergence, and the amplitude of the magnetic field. (Author)

Construction of the high current pulsed gun was completed and the system was used with the superconducting accelerator to generate a high current 24 MeV electron beam. Experiments were conducted with the free electron laser oscillator at 10 microns. The pulsed gun consists of the electron gun and the electronics to drive the gun. The electronics was designed to the gun at a rate corresponding to the round trip optical transit time for the FEL resonator (11.8 MHz) while maintaining the pulse length below 1.5 nsec.

In this report work on a variety of problems involving high energy excitations in solids, instabilities of Fermion systems and phase transistions in 1 and 2 dimensional systems is summarized. A guide is given to publications produced wholly or partially with support from AROD under this grant, which are listed in the appendix. (Author)

Progress attained during the period between 1 October 1972 and 30 September 1974 on the Free Electron Laser Program is reported. The program entails evaluating the feasibility of a new technique for laser excitation leading to tunable, very high-powered lasers at frequency ranges between far infrared and soft x-ray. The technique involves a relativistic electron beam traveling through a transverse, static spatially periodic, magnetic field. The electron beam is supplied by the Stanford Superconducting Linear Accelerator. This report includes a general description of the program, a summary of the scientific background, descriptions of experimental facilities, results to date, and future research plans.

The basic objective of this research was to determine the feasibility of the conversion of the Stanford Mark III linac to high-gradient operation to serve as an injector or driver for storage ring and single pass free electron laser experiments. The Mark III single section tests have been generally successful. In particular, it has been observed that the Mark III accelerator sections are capable of operating reliably and stably at high gradient if an appropriate vacuum is maintained in the structure, and if appropriate steps are taken to bake out the adsorbed contamination on the cavity walls. Electrons have also been accelerated in the test section to an energy in excess of 40 MeV., Personally Identifiable Information (PII) was redacted.

Making use of newly installed experimental hardware and instrumentation, the author has continued his investigation of lase-induced bunch lengthening, gain, diffraction effects, and subthreshold behavior in a ACO storage ring free electron laser. (Author)

The purpose of this research was the identification of the critical magnet and storage ring parameters for optimization of the efficiency, power output and gain of gain-expanded storage ring free electron lasers. While previous research had identified the basic properties of these devices, the approximations and simplifications employed in these efforts for solution of the equations of motion had led to some ambiguities which were not readily resolvable within the framework of the model employed. To resolve these ambiguities, a model was developed for the FEL wiggler magnet which permits an exact solution of the equations of motion. Using this model numerical techniques have been employed to identify the dependence of the laser power output, gain, and efficiency on the magnet parameters., Personally Identifiable Information (PII) was redacted.

Some have described the Food and Drug Administration (FDA) as a scientific bureaucracy with police powers. Does a “cult of infallibility” exist within the FDA, leading to decisions that are contrary to the best interests of patients and their physicians?The Food and Drug Administration (FDA) is one of the most powerful of federal regulatory agencies, if not the most powerful. It regulates over 25% of all consumer goods sold in the United States. It makes decisions on a daily basis that affect the lives of millions of people.While the FDA was created to protect the public, how well is it fulfilling this mission and whose interests is it actually protecting?In this book, four outstanding scholars examine how the FDA accumulated its enormous power and what effects it has had on the public. It also explores who actually benefits and loses from FDA actions, and whether alternatives exist to safeguard the health of Americans. This book raises serious questions about the wisdom of giving policing power to scientists with little oversight or appeal process, as the FDA currently does. It also argues forcefully that the FDA unnecessarily delays beneficial medicines and medical devices, many of which are routinely available in Europe, from being available to Americans.

Some have described the Food and Drug Administration (FDA) as a scientific bureaucracy with police powers. Does a “cult of infallibility” exist within the FDA, leading to decisions that are contrary to the best interests of patients and their physicians?The Food and Drug Administration (FDA) is one of the most powerful of federal regulatory agencies, if not the most powerful. It regulates over 25% of all consumer goods sold in the United States. It makes decisions on a daily basis that affect the lives of millions of people.While the FDA was created to protect the public, how well is it fulfilling this mission and whose interests is it actually protecting?In this book, four outstanding scholars examine how the FDA accumulated its enormous power and what effects it has had on the public. It also explores who actually benefits and loses from FDA actions, and whether alternatives exist to safeguard the health of Americans. This book raises serious questions about the wisdom of giving policing power to scientists with little oversight or appeal process, as the FDA currently does. It also argues forcefully that the FDA unnecessarily delays beneficial medicines and medical devices, many of which are routinely available in Europe, from being available to Americans.

Some have described the Food and Drug Administration (FDA) as a scientific bureaucracy with police powers. Does a “cult of infallibility” exist within the FDA, leading to decisions that are contrary to the best interests of patients and their physicians?The Food and Drug Administration (FDA) is one of the most powerful of federal regulatory agencies, if not the most powerful. It regulates over 25% of all consumer goods sold in the United States. It makes decisions on a daily basis that affect the lives of millions of people.While the FDA was created to protect the public, how well is it fulfilling this mission and whose interests is it actually protecting?In this book, four outstanding scholars examine how the FDA accumulated its enormous power and what effects it has had on the public. It also explores who actually benefits and loses from FDA actions, and whether alternatives exist to safeguard the health of Americans. This book raises serious questions about the wisdom of giving policing power to scientists with little oversight or appeal process, as the FDA currently does. It also argues forcefully that the FDA unnecessarily delays beneficial medicines and medical devices, many of which are routinely available in Europe, from being available to Americans.

Some have described the Food and Drug Administration (FDA) as a scientific bureaucracy with police powers. Does a “cult of infallibility” exist within the FDA, leading to decisions that are contrary to the best interests of patients and their physicians?The Food and Drug Administration (FDA) is one of the most powerful of federal regulatory agencies, if not the most powerful. It regulates over 25% of all consumer goods sold in the United States. It makes decisions on a daily basis that affect the lives of millions of people.While the FDA was created to protect the public, how well is it fulfilling this mission and whose interests is it actually protecting?In this book, four outstanding scholars examine how the FDA accumulated its enormous power and what effects it has had on the public. It also explores who actually benefits and loses from FDA actions, and whether alternatives exist to safeguard the health of Americans. This book raises serious questions about the wisdom of giving policing power to scientists with little oversight or appeal process, as the FDA currently does. It also argues forcefully that the FDA unnecessarily delays beneficial medicines and medical devices, many of which are routinely available in Europe, from being available to Americans.