Researchers at the CNRS Laboratoire d’enzymologie et biochimie structurales, studying Huntington’s disease in collaboration with Professor Ron Kopito’s team at Stanford University, have shown that the normal form of huntingtin protein can acquire an abnormal form without any modification of its genetic code.
These researchers observed that clumps of abnormal huntingtin protein, characteristic of Huntington’s disease, could induce clumping in the normal form of the protein. This work is published in the February 2009 edition of Nature Cell Biology.
Huntington’s disease is a genetic neurological disorder causing neuron degeneration, which in turn affects motor and cognitive functions. The illness arises due to an alteration in the gene sequence coding for huntingtin protein. When Huntington’s disease develops, huntingtin protein forms clumps that hinder normal functions and are closely linked to neurodegeneration.
Researchers at the CNRS Laboratoire d’enzymologie et biochimie structurales, in collaboration with researchers at Stanford University, have shown that huntingtin protein clumps are released from the cells where they develop and can propagate to healthy cells. Once cells are infected, the normal form of huntingtin then starts to clump and the illness spreads. The researchers noticed that the clumps persisted over several generations of cells expressing normal huntingtin following their temporary exposure to protein clumps from Huntington’s disease. This contamination by proximity is similar to the development of illnesses caused by prions (encephalopathies associated with "abnormal" prions).
These results suggest that huntingtin protein clumps are transmissible and that their propagation from one cell to another could be a generic vector of neurodegenerative illnesses.Full bibliographic information:
Julien Guillaume | alfa
Immune Defense Without Collateral Damage
23.01.2017 | Universität Basel
The interactome of infected neural cells reveals new therapeutic targets for Zika
23.01.2017 | D'Or Institute for Research and Education
For the first time ever, a cloud of ultra-cold atoms has been successfully created in space on board of a sounding rocket. The MAIUS mission demonstrates that quantum optical sensors can be operated even in harsh environments like space – a prerequi-site for finding answers to the most challenging questions of fundamental physics and an important innovation driver for everyday applications.
According to Albert Einstein's Equivalence Principle, all bodies are accelerated at the same rate by the Earth's gravity, regardless of their properties. This...
An important step towards a completely new experimental access to quantum physics has been made at University of Konstanz. The team of scientists headed by...
Yersiniae cause severe intestinal infections. Studies using Yersinia pseudotuberculosis as a model organism aim to elucidate the infection mechanisms of these...
Researchers from the University of Hamburg in Germany, in collaboration with colleagues from the University of Aarhus in Denmark, have synthesized a new superconducting material by growing a few layers of an antiferromagnetic transition-metal chalcogenide on a bismuth-based topological insulator, both being non-superconducting materials.
While superconductivity and magnetism are generally believed to be mutually exclusive, surprisingly, in this new material, superconducting correlations...
Laser-driving of semimetals allows creating novel quasiparticle states within condensed matter systems and switching between different states on ultrafast time scales
Studying properties of fundamental particles in condensed matter systems is a promising approach to quantum field theory. Quasiparticles offer the opportunity...
19.01.2017 | Event News
10.01.2017 | Event News
09.01.2017 | Event News
23.01.2017 | Health and Medicine
23.01.2017 | Physics and Astronomy
23.01.2017 | Process Engineering