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 World first: Massive thrombosis removed during early pregnancy
20.07.2017 | Universitätsspital Bern

nachricht Therapy of preterm birth in sight?
19.07.2017 | Universitätsspital Bern

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: Manipulating Electron Spins Without Loss of Information

Physicists have developed a new technique that uses electrical voltages to control the electron spin on a chip. The newly-developed method provides protection from spin decay, meaning that the contained information can be maintained and transmitted over comparatively large distances, as has been demonstrated by a team from the University of Basel’s Department of Physics and the Swiss Nanoscience Institute. The results have been published in Physical Review X.

For several years, researchers have been trying to use the spin of an electron to store and transmit information. The spin of each electron is always coupled...

Im Focus: The proton precisely weighted

What is the mass of a proton? Scientists from Germany and Japan successfully did an important step towards the most exact knowledge of this fundamental constant. By means of precision measurements on a single proton, they could improve the precision by a factor of three and also correct the existing value.

To determine the mass of a single proton still more accurate – a group of physicists led by Klaus Blaum and Sven Sturm of the Max Planck Institute for Nuclear...

Im Focus: On the way to a biological alternative

A bacterial enzyme enables reactions that open up alternatives to key industrial chemical processes

The research team of Prof. Dr. Oliver Einsle at the University of Freiburg's Institute of Biochemistry has long been exploring the functioning of nitrogenase....

Im Focus: The 1 trillion tonne iceberg

Larsen C Ice Shelf rift finally breaks through

A one trillion tonne iceberg - one of the biggest ever recorded -- has calved away from the Larsen C Ice Shelf in Antarctica, after a rift in the ice,...

Im Focus: Laser-cooled ions contribute to better understanding of friction

Physics supports biology: Researchers from PTB have developed a model system to investigate friction phenomena with atomic precision

Friction: what you want from car brakes, otherwise rather a nuisance. In any case, it is useful to know as precisely as possible how friction phenomena arise –...

All Focus news of the innovation-report >>>

Anzeige

Anzeige

Event News

Closing the Sustainability Circle: Protection of Food with Biobased Materials

21.07.2017 | Event News

»We are bringing Additive Manufacturing to SMEs«

19.07.2017 | Event News

The technology with a feel for feelings

12.07.2017 | Event News

 
Latest News

NASA looks to solar eclipse to help understand Earth's energy system

21.07.2017 | Earth Sciences

Stanford researchers develop a new type of soft, growing robot

21.07.2017 | Power and Electrical Engineering

Vortex photons from electrons in circular motion

21.07.2017 | Physics and Astronomy

VideoLinks
B2B-VideoLinks
More VideoLinks >>>