Forum for Science, Industry and Business

Sponsored by:     3M 
Search our Site:

 

Researchers Say Deadly Twist Key To Sickle Cell Disease

31.03.2003


Patients with sickle cell disease have mutant haemoglobin proteins that form deadly long, stiff fibres inside red blood cells. A research team led by University of Warwick researcher Dr Matthew Turner, propose a mathematical model in the 28 March online issue of PRL to explain the persistent stability of these deadly fibres. The theory suggests that an inherent "twistiness" in the strands that make up the fibres could be the key to their durability and possibly to new treatments.



Red blood cells supply oxygen to the body using their cargo of haemoglobin, a protein that can capture and release oxygen. Haemoglobin molecules normally float freely in the cell, but sickle cell patients have a mutated, "sticky," form of haemoglobin that tends to clump together into long fibres. The stiff fibres form a scaffolding that distorts the cells into their namesake "sickle" shape, so they jam up trying to pass through small blood vessels. The traffic jams deprive vital organs of oxygen, so patients end up with anaemia, jaundice, major organ damage, and many other maladies.

A sickle haemoglobin fibre can be made up of anywhere from 14 to more than 400 individual strands of haemoglobin molecules linked into long chains. Matthew Turner, of the University of Warwick in the UK, wondered why these strands tend to clump together into long, stiff, fibres rather than compact crystals, which would be less harmful. "A scaffolding made of the rigid fibres is much worse than a couple little sugar-cube-like crystals floating around," Turner says. So he and his colleagues constructed a mathematical model.


The team’s equations start with the trade-offs that exist in any material as it tries to find the shape with the least overall stress. The forces at work include bending and stretching, and for haemoglobin strands, there is also a propensity to stick together. This stickiness would normally make a thick, compact crystal more stable than a thin fibre, Turner explains, because a crystal maximizes the contact area of the protein with itself. But for sickle haemoglobin, fibres are more stable. To favour fibres, the equations needed to include the fact that the individual strands of molecules are inherently "twisty." They behave like the coiled wire that attaches a telephone to its handset, apparently because the molecules link up in a way that favours twisting. The strands wrap around one another like threads of rope to form the fibres. In their paper, the team shows that their model’s predictions for two of the mechanical properties of fibres agree with experiments.

Turner says that the model suggests a possible treatment for sickle cell disease. Gene therapy could introduce a haemoglobin mutant that formed less-twisty individual strands, and this "good mutant" might turn fibres into less harmful crystals. Simply introducing normal haemoglobin has been shown not to work, perhaps because the few normal haemoglobin molecules cannot eliminate the fibres.

Peter Dunn | alfa
Further information:
http://www.communicate.warwick.ac.uk/index.cfm?page=pressrelease&id=973

More articles from Health and Medicine:

nachricht Hot cars can hit deadly temperatures in as little as one hour
24.05.2018 | Arizona State University

nachricht 3D images of cancer cells in the body: Medical physicists from Halle present new method
16.05.2018 | Martin-Luther-Universität Halle-Wittenberg

All articles from Health and Medicine >>>

The most recent press releases about innovation >>>

Die letzten 5 Focus-News des innovations-reports im Überblick:

Im Focus: Powerful IT security for the car of the future – research alliance develops new approaches

The more electronics steer, accelerate and brake cars, the more important it is to protect them against cyber-attacks. That is why 15 partners from industry and academia will work together over the next three years on new approaches to IT security in self-driving cars. The joint project goes by the name Security For Connected, Autonomous Cars (SecForCARs) and has funding of €7.2 million from the German Federal Ministry of Education and Research. Infineon is leading the project.

Vehicles already offer diverse communication interfaces and more and more automated functions, such as distance and lane-keeping assist systems. At the same...

Im Focus: Molecular switch will facilitate the development of pioneering electro-optical devices

A research team led by physicists at the Technical University of Munich (TUM) has developed molecular nanoswitches that can be toggled between two structurally different states using an applied voltage. They can serve as the basis for a pioneering class of devices that could replace silicon-based components with organic molecules.

The development of new electronic technologies drives the incessant reduction of functional component sizes. In the context of an international collaborative...

Im Focus: LZH showcases laser material processing of tomorrow at the LASYS 2018

At the LASYS 2018, from June 5th to 7th, the Laser Zentrum Hannover e.V. (LZH) will be showcasing processes for the laser material processing of tomorrow in hall 4 at stand 4E75. With blown bomb shells the LZH will present first results of a research project on civil security.

At this year's LASYS, the LZH will exhibit light-based processes such as cutting, welding, ablation and structuring as well as additive manufacturing for...

Im Focus: Self-illuminating pixels for a new display generation

There are videos on the internet that can make one marvel at technology. For example, a smartphone is casually bent around the arm or a thin-film display is rolled in all directions and with almost every diameter. From the user's point of view, this looks fantastic. From a professional point of view, however, the question arises: Is that already possible?

At Display Week 2018, scientists from the Fraunhofer Institute for Applied Polymer Research IAP will be demonstrating today’s technological possibilities and...

Im Focus: Explanation for puzzling quantum oscillations has been found

So-called quantum many-body scars allow quantum systems to stay out of equilibrium much longer, explaining experiment | Study published in Nature Physics

Recently, researchers from Harvard and MIT succeeded in trapping a record 53 atoms and individually controlling their quantum state, realizing what is called a...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

VideoLinks
Industry & Economy
Event News

In focus: Climate adapted plants

25.05.2018 | Event News

Save the date: Forum European Neuroscience – 07-11 July 2018 in Berlin, Germany

02.05.2018 | Event News

Invitation to the upcoming "Current Topics in Bioinformatics: Big Data in Genomics and Medicine"

13.04.2018 | Event News

 
Latest News

In focus: Climate adapted plants

25.05.2018 | Event News

Flow probes from the 3D printer

25.05.2018 | Machine Engineering

Less is more? Gene switch for healthy aging found

25.05.2018 | Life Sciences

VideoLinks
Science & Research
Overview of more VideoLinks >>>