Cardiopulmonary function in two human disorders of the hypoxia-inducible factor (HIF) pathway: von Hippel-Lindau disease and HIF-2 alpha gain-of-function mutation

The hypoxia-inducible factors (HIFs; isoforms HIF-1 alpha, HIF-2 alpha, HIF-3 alpha) mediate many responses to hypoxia. Their regulation is principally by oxygen-dependent degradation, which is initiated by hydroxylation of specific proline residues followed by binding of von Hippel-Lindau (VHL) protein. Chuvash polycythemia is a disorder with elevated HIF. It arises through germline homozygosity for hypomorphic VHL alleles and has a phenotype of hematological, cardiopulmonary, and metabolic abnormalities. This study explores the phenotype of two other HIF pathway diseases: classic VHL disease and HIF-2 alpha gain-of-function mutation. No cardiopulmonary abnormalities were detected in classic VHL disease. HIF-2 alpha gain-of-function mutations were associated with pulmonary hypertension, increased cardiac output, increased heart rate, and increased pulmonary ventilation relative to metabolism. Comparison of the HIF-2 alpha gain-of-function responses with data from studies of Chuvash polycythemia suggested that other aspects of the Chuvash phenotype were diminished or absent. In classic VHL disease, patients are germline heterozygous for mutations in VHL, and the present results suggest that a single wild-type allele for VHL is sufficient to maintain normal cardiopulmonary function. The HIF-2 alpha gain-of-function phenotype may be more limited than the Chuvash phenotype either because HIF-1 alpha is not elevated in the former condition, or because other HIF-independent functions of VHL are perturbed in Chuvash polycythemia.-Formenti, F., Beer, P. A., Croft, Q. P. P., Dorrington, K. L., Gale, D. P., Lappin, T. R. J., Lucas, G. S., Maher, E. R., Maxwell, P. H., McMullin, M. F., O'Connor, D. F., Percy, M. J., Pugh, C. W., Ratcliffe, P. J., Smith, T. G., Talbot, N. P., Robbins, P. A. Cardiopulmonary function in two human disorders of the hypoxia-inducible factor (HIF) pathway: von Hippel-Lindau disease and HIF-2 alpha gain-of-function mutation. FASEB J. 25, 2001-2011 (2011). www.fasebj.org

One Dimensional Modeling of a Turbogenerating Spark Ignition Engine Operating on Biogas

Turbocompounding is generally regarded as the process of recovering a proportion of the exhaust gas energy from a reciprocating engine and applying it to the output power of the crankshaft. In conventional turbocompounding, the power turbine has been mechanically connected to the crankshaft but now a new method has emerged. Recent advances in high speed electrical machines have enabled the power turbine to be coupled to an electric generator. Decoupling the power turbine from the crankshaft and coupling it to a generator allows the power electronics to control the turbine speed independently in order to optimize the turbine efficiency for different engine operating conditions.

The technical merits of turbogenerating shown through the design, validation and implementation of a 1 dimensional engine model

Turbocompounding is the process of recovering a proportion of an engine’s fuel energy that would otherwise be lost in the exhaust process and adding it to the output power. This was first seen in the 1930s and is carried out by coupling an exhaust gas turbine to the crankshaft of a reciprocating engine. It has since been recognised that coupling the power turbine to an electrical generator instead of the crankshaft has the potential to reduce the fuel consumption further with the added flexibility of being able to decide how this recovered energy is used. The electricity generated can be used in automotive applications to assist the crankshaft using a flywheel motor generator or to power ancillaries that would otherwise have run off the crankshaft. In the case of stationary power plants, it can assist the electrical power output. Decoupling the power turbine from the crankshaft and coupling it to a generator allows the power electronics to control the turbine speed independently in order to optimise the specific fuel consumption for different engine operating conditions. This method of energy recapture is termed ‘turbogenerating’.

This paper gives a brief history of turbocompounding and its thermodynamic merits. It then moves on to give an account of the validation of a turbogenerated engine model. The model is then used to investigate what needs to be done to an engine when a turbogenerator is installed. The engine being modelled is used for stationary power generation and is fuelled by an induced biogas with a small portion of palm oil being injected into the cylinder to initiate combustion by compression ignition. From these investigations, optimum settings were found that result in a 10.90% improvement in overall efficiency. These savings relate to the same engine without a turbogenerator installed operating with fixed fuelling.

Investigations Into the Performance of a Turbogenerated Biogas Engine During Speed Transients

Turbogenerating is a form of turbocompounding whereby a Turbogenerator is placed in the exhaust stream of an internal combustion engine. The Turbogenerator converts a portion of the expelled energy in the exhaust gas into electricity which can then be used to supplement the crankshaft power. Previous investigations have shown how the addition of a Turbogenerator can increase the system efficiency by up to 9%. However, these investigations pertain to the engine system operating at one fixed engine speed. The purpose of this paper is to investigate how the system and in particular the Turbogenerator operate during engine speed transients. On turbocharged engines, turbocharger lag is an issue. With the addition of a Turbogenerator, these issues can be somewhat alleviated. This is done by altering the speed at which the Turbogenerator operates during the engine’s speed transient. During the transients, the Turbogenerator can be thought to act in a similar manner to a variable geometry turbine where its speed can cause a change in the turbocharger turbine’s pressure ratio. This paper shows that by adding a Turbogenerator to a turbocharged engine the transient performance can be enhanced. This enhancement is shown by comparing the turbogenerated engine to a similar turbocharged engine. When comparing the two engines, it can be seen that the addition of a Turbogenerator can reduce the time taken to reach full power by up to 7% whilst at the same time, improve overall efficiency by 7.1% during the engine speed transient.

Introducing the CTA concept

The Cherenkov Telescope Array (CTA) is a new observatory for very high-energy (VHE) gamma rays. CTA has ambitions science goals, for which it is necessary to achieve full-sky coverage, to improve the sensitivity by about an order of magnitude, to span about four decades of energy, from a few tens of GeV to above 100 TeV with enhanced angular and energy resolutions over existing VHE gamma-ray observatories. An international collaboration has formed with more than 1000 members from 27 countries in Europe, Asia, Africa and North and South America. In 2010 the CTA Consortium completed a Design Study and started a three-year Preparatory Phase which leads to production readiness of CTA in 2014. In this paper we introduce the science goals and the concept of CTA, and provide an overview of the project. © 2013 Elsevier B.V. All rights reserved.