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In a pilot study of two patients monitored for two years, an international team of researchers slowed the onset of the debilitating brain disease X-linked adrenoleukodystrophy (ALD) using a lentiviral vector to introduce a therapeutic gene into patient’s blood cells. Although studies with larger cohorts of patients are needed, these results suggest that gene therapy with lentiviral vectors, which are derived from disabled versions of human immunodeficiency virus (HIV), could potentially become instrumental in treating a broad range of human disorders

Other Gene Therapy Success and Progress
Lungs too damaged for use in transplant operations may be salvageable through a gene-based technique, doubling or tripling the supply of organs.

The flawed lungs could be removed from donors’ bodies after death and repaired using the gene IL-10, which lowers inflammation. 1800 people in the US are awaiting lung transplants.

Gene Therapy helps treat a form of blindness The condition is known as Leber’s congenital amaurosis and there are 2000 people in the US who have it.

A number of companies are developing gene therapies and 320 trials are under way or cleared to begin by U.S. regulators, said Karen Riley, a U.S. Food and Drug Administration spokeswoman. Genzyme Corp. of Cambridge, Massachusetts, will begin a human trial using gene therapy next year to treat macular degeneration, the leading form of age-related vision loss, said John Lacey, a Genzyme spokesman

Researchers at the California Institute of Technology (Caltech) have shown that a highly specific intrabody (an antibody fragment that works against a target inside a cell) is capable of stalling the development of Huntington’s disease in a variety of mouse models.

Research in monkeys suggests that genetically delivering dopamine avoids some side effects and helps with Parkinson’s.

In the new trial, reported today in the journal Science Translational Medicine, Bechir Jarraya and colleagues at the Molecular Imaging Research Center in Fontenay-aux-Roses, France, mimicked Parkinson’s in monkeys by giving them a neurotoxin that causes movement problems characteristic of the disorder. The researchers then injected three genes involved in dopamine production into the brains of the monkeys, as well as specially designed probes to measure dopamine levels in the brain, monitoring the animals for up to three and a half years. The gene therapy restored concentrations of dopamine in the brain, corrected movement problems, and prevented dyskinesias–without any severe adverse side effects. An early stage human clinical trial using the same dopamine gene therapy approach is now underway.

The Modified HIV Gene Therapy

The healthy ALD protein was expressed in about 15 percent of blood cells, yet surprisingly this low level was sufficient to slow brain disease in ALD. “This percentage of correction will not be sufficient for all diseases,” warns Aubourg. “There is a lot of work to be done to make this gene therapy vector more powerful, less complicated, and less expensive. This is only the beginning,” he said.

Gene therapy is not without serious risks. Like other retrovirus vectors, the HIV-derived lentivirus vector is tasked with inserting the therapeutic gene in the chromosomes of the patients’ cells. In a worst case scenario, this action could disturb the biology of the cells and patients could end up with leukemia; this outcome has occurred in past gene therapy trials. “The HIV-derived lentivirus vector basically has this same risk, although the design of the vector makes patients less prone to this side effect,” said Aubourg.

Wrong Diagnosis has statistics on ALD

Prevalance Rate: approx 1 in 20,000 or 0.00% or 13,600 people in USA

Wikipedia on Adrenoleukodystrophy

Adrenoleukodystrophy (ALD) (also known as “Addison-Schilder Disease,” “Siemerling-Creutzfeldt Disease,” and “Schilder’s disease”) is a rare, inherited disorder that leads to progressive brain damage, failure of the adrenal glands and eventually death. ALD is one disease in a group of inherited disorders called leukodystrophies. Adrenoleukodystrophy progressively damages the myelin, a complex fatty neural tissue that insulates many nerves of the central and peripheral nervous systems, eventually destroying it. Without myelin, nerves are unable to conduct an impulse, leading to increasing disability as myelin destruction increases and intensifies.

FURTHER READING

NY Times: For Gene Therapy, Seeing Signs of a Resurgence

Lawrenceville Plasma Physics (LPP) a small research and development company part way through a two-year-long experimental project to test the scientific feasibility of Focus Fusion, controlled nuclear fusion using the dense plasma focus (DPF) device and hydrogen-boron fuel. Hydrogen-boron fuel produces almost no neutrons and allows the direct conversion of energy into electricity. Success would mean thousands of times more total energy would be available and the energy would be cleaner and cheaper. LPP believes that with success they can lower the cost of energy up to 50 times.

This site has described how the Mr. Fusion scenario would change the world.

They have achieved one of eight experimental goals so far. The eight goals and the timeline they are working on as listed at FocusFusion.org.

By the End of 2009

* At 25kV (kilovolts): Produce 1 MA (million amperes), determine optimum gas pressure

Get the experimental machine to function at 25 kilovolts, the lowest planned experimental voltage, and to produce more than 1 Million Amps of current. They will also very shortly switch over to running with deuterium and thus achieve their first fusion reactions with FF-1. In achieving this goal, they will also determine the optimum gas pressure for this current.

* Test theory of axial magnetic field

The third goal is to test the theory that adding a small axial magnetic field, and thus a small amount of angular momentum, to the plasma will greatly increase the size of the plasmoids and thus the efficiency of energy transfer into the plasmoid.

* Move to 45kV, 2MA, with Deuterium
The fourth goal is to increase the charging potential on the machine, by 5 kV steps, up to the full capacity of 45 kV and in the process achieve a peak current of about 2 MA with deuterium.

* Confirm University of Texas Dense Plasma Fusion results, with better instruments

The fifth goal is to confirm the Texas results of high temperature and density, but with far more complete diagnostic instruments.

By end of 2010

* Heavier Gases: D + He + N, and shorter electrodes

The sixth goal is to confirm LPP’s theory that heavier gases will lead to higher compression and to thereby achieve gigagauss fields. This will involve running with combination of D (deteurium), He (Helium) and perhaps N (Nitrogen) and will also involve replacing the electrode with shorter ones, which they predict will be optimized for the heavier gases. These experiments are more complex and will be more time-consuming.

* pB11

The seventh goal is to demonstrate some fusion burn with pB11 (proton-boron) fuel.

Proton-boron fusion would have very little neutron radiation as described in the wikipedia entry on aneutronic fusion

* Net energy
The eighth and final goal will be to demonstrate the scientific feasibly of producing net energy with pB11.

First Goal Achieving a Pinch, Has Been Done. Why it Matters

From Focus Fusion, Eric Lerner summarizes the significance of first shots and pinch as follows:

The achievement of a pinch, and on the second shot, means that we have accomplished one of the eight technical goals of the current experimental program. The machine is doing what we designed it to do, which is to transfer energy into a tiny plasmoid. It is quite unusual for a DPF to pinch right way. Normally fine-tuning of the electrodes and insulator and “conditioning” of the electrodes by several shots is required. That this was not needed is confirmation that our electrode and insulator dimensions, derived from LPP’s quantitative theory of DPF functioning, are accurate.

Tweaking of the Experimental System to Setup for Firing/Shots

The “down time” the crew has been experiencing stems from various components in the machine which prevent the “shot” from going off as it should. The whole machine, in a sense, has to be fine tuned to eliminate leaks and losses and bring the charge to bear along the electrodes with the correct timing, and keep the gas in the vacuum.

Various components such as the vacuum, switches, triggers and so forth have been assembled, disassembled, tweaked, re-assembled.

Consider the vacuum chamber. It has many vulnerable points – there are “windows” for observation and connecting diagnostic instruments. Each connection point represents some vulnerability. Every time they change something, they have to test the vacuum again. There’s a big table in the room with FoFu, covered with tools. I visit the lab, and the guys are in there, switching out a rogowski coil from the drift tube, for example. Re-connecting it. Testing the vacuum again. This is why the machine was designed as it is, with access to walk in under the machine and constantly take things off and add things on.

Robotic fish will use electro-active-polymer fins to gather precise data on aquatic conditions and habitats, in hopes of gaining answers about the effects of climate change and other outside forces on our freshwater ecosystems. Look for widespread use of robotic fish for civilian and military applications within five years. R.C.J.

Robo-fish will soon be flexing their electro-active-polymer fins to perform critical environmental monitoring that safeguards our lakes, aqua farms and reservoirs.
Robotic fish are needed to monitor the quality of water in rivers, streams and lakes, according to Michigan State University researchers who have crafted artificial muscles to power them. Electro-active polymers use electrical signals from a microprocessor to activate artificial muscles that enable the robots to swim in a manner similar to natural fish. Watch a video showing how electrical engineer Xiaoba Tan is collaborating with zoologist Elema Litchman to design their robo-fish
Text: http://www.smartertechnology.com/c/a/Technology-For-Change/RoboFish-to-Perform-Smarter-Water-Monitoring/

Guest post by Gary Nevison, Farnell

Summary
The legal obligations within the REACH Regulations and the potential impact of the so called “RoHS2” proposals dominate the legislative scene at present. Also, obligations are starting to filter through as part of the eco design/energy using Products Directive that monitors energy efficiency throughout a products life cycle. Going-forward, the scope of this directive will become more diverse to encompass energy related products.

Industry, and the design engineer, needs to be aware of developments and the potential impact on their companies.

REACH
Regulation 1907/2006, the Regulation, Evaluation, Authorisation -and restriction-of Chemicals (REACH) entered into force on 1 June 2007 after almost 8 years of debate and covering some 849 pages of regulation.

The only census on chemicals, taken in 1981, highlighted that 100,106 substances were placed on the market. Of those used, manufactured or imported at levels of 1000 tonnes or more, 21 percent had no safety data at all while a further 65 percent provided insufficient safe use data. Only 3 percent had been fully tested.

At the same time, instances of allergies, asthma, certain types of cancer and reproductive disorders were on the increase in Europe. Skin diseases alone resulted in the loss of 3 million working days per year.

Driven by the obligatory flow of safety data throughout the supply chain, REACH seeks to provide protection to health and the environment.

The biggest challenge to industry to date has been around the collection of data advising where a so called Substance of Very High Concern (SVHC) is present in a product, known as an article in the regulations, at a level of 0.1% by weight of the total article.

As a minimum obligation the name of the substance along with supporting safe use data must flow downstream through the supply chain.

Substances that are categorised as SVHC include those that are carcinogenic, mutagenic or toxic for reproduction. Also, substances that are persistent, liable to bio-accumulate and toxic, and finally “others” such as endocrine disrupters that can have a particular impact on aquatic life.

Back in October 2008, the first batch of 15 substances of very high concern was published by the European Chemicals Agency and, the following January, seven of these were earmarked for a consultation on whether or not they should be subject to a very costly “authorisation of use” requirement. These seven were approved and the European Commission will determine the date when these will be added to Annex XIV.

These obligations will then enter into force some 42-48 months later on what is known as the sunset date. Where such an authorisation is approved, downstream users can only use these substances for the use that they have been authorised for and they can only be purchased from the company granted the authorisation.

The REACH data collection has resulted in the circulation of hundreds of “standard letters” in a multitude of formats. Many manufacturers have refused to reply to the ad-hoc requests they receive preferring to promote websites or central databases (these have been slow to develop).

Guidance on the regulation encourages a more pro-active approach rather than simply providing a link to a website. Likewise, there have been many random requests for information that is not obligatory such as pre-registration details and RoHS style certificates of compliance.

Future batches of SVHCs will be published on an on-going basis, with the next expected early 2010, so REACH will not be a box that can be ticked as complete for several years to come.

“RoHS2”
Looking back the Restriction of Hazardous Substances (RoHS) Directive 2002/95 entered into force on 1 July 2006. It featured six restricted substances across eight broad categories of product pulled from the 10 categories in the Waste Electrical and Electronic Equipment (WEEE) Directive. There were 29 exemptions to assist manufacturers and design engineers where no viable alternative was available and a whole raft of “grey” definitions that required clarity.

“RoHS2” proposals look to move the directive forward and provide greater clarity. However, some of its provisions will potentially have cost and resource issues for industry.

The proposals recommend that the two remaining categories from the original WEEE categories, namely medical devices and monitoring and control instruments be added to the scope from 2014 (in-vitro diagnostics from 2016 and industrial “test” equipment in 2017). These were originally omitted from the directive due to reliability concerns over the use of lead-free solder.

While there are no substances actually restricted under the proposals, four are recommended for priority assessment. Three plasticisers used in a variety of applications and a flame retardant may well be restricted. Ironically, the substances, BBP, DBP, DEHP and HBCDD are four of the seven subject to authorisation of use under the REACH Regulations.

The fate of these substances under RoHS will be open to consultation after the revised directive comes into scope sometime in 2011-2012. Either way, any restriction under RoHS will probably be sooner than under the REACH process and the analysis is unlikely to be done twice.

Under a separate review by European Commission consultants, 29 exemptions will continue under the proposals, many with amended wording for clarity, 6 will be withdrawn and one new one will be granted. These could come into force next year and that will be followed by a transposition period of, on average, 18 months allowing manufacturers the time to comply.

In addition, a further six exemptions were added in June 2009 that had been proposed a year earlier.

RoHS2 also clarifies definitions such as equipment within out of scope equipment, spare parts and military where the latter clearly does not include dual use equipment.

A standard, and rigid declaration of conformity appears in annex 7 and will replace the multitude of different certificates, statements and compliance documents under the original legislation.

There now appears to be no scope for qualifying statements such as “so far as we are aware” and “to the best of our knowledge”.

It is proposed that RoHS will become a CE mark directive placing responsibilities on manufacturers, importers and distributors. There are many requirements including building technical files and keeping for 10 years, ensuring products comply, that they are supplied with the CE mark and the manufacturer or importer is identified on the product. Sample testing should also be carried out where appropriate, and corrective action is undertaken where product is found to be non compliant.

Finally under the proposals, the broad product categories and list of indicative products move from the WEEE Directive and sit in annex 1 and 2 of the RoHS Directive.

The terminology “RoHS2” is unofficial and is used simply to highlight the proposed changes to the original directive.

Energy using Products
Directive 2005/32/EC, Energy using Products, entered into force on 11 August 2007.

The main aim is to monitor energy efficiency throughout the life cycle of a product from the mining of raw material right through to recycling at end-of-life.

The focus is clearly on the design phase of the product since it is considered that this is the determining stage affecting the resources used in a product.

The directive does not apply to means of transport (planes, cars etc) but, apart from this, the scope is deliberately broad covering, in principle, any product which when in use depends on, generates, transfers or measures energy (electricity, fossil fuel or renewable).

Obligations on manufacturers will result from a series of specific “implementing measures” the first of which was adopted in January 2009.

The criteria required before a product can be considered for assessment is that is that it must sell more than 200,000 units per year in the European Union, have a significant environmental impact and present considerable potential for improvement. The latter is important as the target is for a 20% improvement in energy efficiency by 2020.

During phase one, studies were commission on 20 broad product categories ranging from water heaters, televisions and lighting, through to imaging equipment and personal computers.

Beyond that, a further 17 categories have been highlighted from ovens and hobs, machine tools and air-conditioning equipment through to transformers and networking equipment.

Among the first obligations to come in to force were energy efficiency improvements covering standby and off-mode losses, simple converter boxes for digital television, external power supplies and office, street and domestic lighting.

However, the European Parliament has now adopted the European Commission’s (EC) proposal to widen the scope of the directive to include energy related products. Until now the Energy using Products Directive was limited to products that consume energy during use such as boilers, computers, televisions, industrial fans and light bulbs.

However, many products have an indirect impact on the energy in use such as water using devices, taps and showerheads for example, and double glazing windows or insulating material.

Improvement in design could clearly result in the significant saving of energy.

For example, water saving taps and shower heads reduce water consumption and therefore the amount of energy used for hot water.

Under the EuP Directive studies had to set requirements for individual products where, in fact, it is the performance of the whole system that often needs to be optimised not just a single component or products.

The new directive will repeal the existing 2005/32/EC.

Intel will use its upcoming Intel Developer Forum to highlight several key developments in its product roadmap — starting with smaller, more efficient chip designs and specialized applications for its Nehalem line.

* Intel will show off “Westmere,” the first processors built using a 32 nanometer (nm) manufacturing process.
* CPU manufacturing shrank from 65nm to 45nm to 32nm and next to 22nm.
* The next chip architecture will come in 2010, in the form of the new architecture codenamed “Sandy Bridge,” which will also be disclosed at this month’s IDF. Intel’s roadmap is process shrinkage and then better architecture and then process shrink etc… (tick-tock)
* This new-generation high-k metal gate transistor formula will give Intel “a 3+ year advantage in addressing leaky and energy inefficient transistors,” according to a blog post from Intel spokesman Bill Kircos Intel has shipped >200 million 45nm CPUs using high-k+ metal gate transistors.
* For the first time, Intel has developed a full-featured SoC process technology to complement the CPU-specific technology. This version is for our smarter System on Chip (SoC) product efforts, which emphasize lower power transistors
* Intel NMOS transistors now have 19% performance improvement over their 45nm counterparts and our PMOS transistors now have a 28% performance improvement over their 45nm counterparts.
* Another IDF highlight: Nehalem-based chips codenamed “Jasper Forest” and designed for the embedded and storage sectors. This family of products will bring Nehalem to the embedded market, offering integrated PCI Express (PCIe) and an integrated I/O hub in a dual-processor Xeon processor.
* Nehalem will allow for much faster and denser storage and communications solutions such as IPTV, VoIP, NAS, SAN and wireless radio network controllers

The UK Register has information on the Sun Sparc Roadmap. The 16-core “Rock” UltraSparc-RK processor for Sun’s once-and-never “Supernova” line of servers is not on the roadmap. The one-page roadmap is one given Sun’s customers – and presumably also Fujitsu’s customers – have been shown about the future Sparc processor lineup.

June 2009 Sun Roadmap

A long way off in late 2010 or early 2011, the Sparc Enterprise server lineup gets a speed boost to 3 GHz with the Jupiter-E chips.

After that, in 2012, Sun has made no commitment to the kicker line of Fujitsu “Advanced Product Line 2″ servers coming from Fujitsu. These APL2 machines are presumably to be based on the “Venus” eight-core Sparc64-VIII processor, which has a Sparc64-VIIIfx variant aimed at supercomputers. That Sparc64-VIIIfx chip will be used in a 10 petaflops massively parallel machine being built by Fujitsu and paid for by the Japanese government under the 1.2bn Project Keisoku effort.

All of this is subject to change, and some of it most certainly will once Oracle takes control of Sun.