The study is being published September 14 in Science X-Press, an advanced, online edition of the journal Science.
In the study, the researchers suggest that herpes simplex encephalitis may reflect a single gene immunodeficiency that confers susceptibility to herpes simplex virus, an idea that contrasts with the prevailing scientific theory of how genes work to make people vulnerable to infections. These new findings, the study added, may apply to other infectious diseases as well.
In the study, scientists focused on blood cells from two French children with a deficiency for UNC-93B, an endoplasmic reticulum protein involved in the recognition of pathogens. When infected with herpes simplex virus-1, the UNC-93B-deficient cells were unable to produce natural interferons alpha, beta, and gamma (IFNs -?/? and -?). Interferons are produced by the immune system to fight infections and tumors.
This deficiency resulted in high rates of herpes simplex virus-1 proliferation and cell death. Assuming these findings extend to neurons, they provide a plausible mechanism for herpes simplex encephalitis.
"We and our colleagues have identified recessive UNC-93B deficiency as a genetic etiology of herpes simplex encephalitis in otherwise healthy patients," said Professor Bruce Beutler, M.D., one of three Scripps Research scientists who contributed to the study. "The discovery of this genetic cause for herpes simplex encephalitis not only broadens our understanding of these types of immunodeficiencies, but also has important therapeutic implications-some of these patients could benefit from recombinant interferon alpha (IFN-?) treatment, just as patients with low levels of naturally occurring interferon gamma (IFN-?) benefit from a similar life-saving approach."
Herpes simplex virus-1 is a common virus that infects about 80 percent of young adults worldwide. Herpes simplex encephalitis, a viral infection of the brain that affects otherwise healthy patients, affects an extremely small percentage of those infected with herpes simplex virus-1: the number of annual cases is two per million people, according to the University of Maryland Medical Center.
Nonetheless, herpes simplex encephalitis, which was first described in 1941, is the most common type of sporadic viral encephalitis in developed countries, accounting for about 10 to 20 percent of all viral encephalitis cases, according to the University of Maryland Medical Center. Before the advent of anti-viral drugs vidarabine in 1973 and acyclovir in 1981, mortality rates reached up to 70 percent. While the introduction of anti-viral treatment has been a boon to patients, brain damage still poses a substantial risk.
"In contrast to most current thinking, we suspected that herpes simplex encephalitis susceptibility could be inherited as a monogenic trait resulting in the specific impairment of immunity to herpes simplex virus-1," Beutler said. "Here we have defined a genetic lesion that is permissive for an infection: a trait that most would regard as quintessentially environmental in cause. It is likely that other mutations will also be found to permit herpes simplex virus encephalitis, and likely that other infectious diseases will ultimately be traced to mutations that affect UNC-93B."
Keith McKeown | EurekAlert!
New study: How does Europe become a leading player for software and IT services?
03.04.2017 | Fraunhofer-Institut für System- und Innovationsforschung (ISI)
Reusable carbon nanotubes could be the water filter of the future, says RIT study
30.03.2017 | Rochester Institute of Technology
Two-dimensional magnetic structures are regarded as a promising material for new types of data storage, since the magnetic properties of individual molecular building blocks can be investigated and modified. For the first time, researchers have now produced a wafer-thin ferrimagnet, in which molecules with different magnetic centers arrange themselves on a gold surface to form a checkerboard pattern. Scientists at the Swiss Nanoscience Institute at the University of Basel and the Paul Scherrer Institute published their findings in the journal Nature Communications.
Ferrimagnets are composed of two centers which are magnetized at different strengths and point in opposing directions. Two-dimensional, quasi-flat ferrimagnets...
An Australian-Chinese research team has created the world's thinnest hologram, paving the way towards the integration of 3D holography into everyday...
In the race to produce a quantum computer, a number of projects are seeking a way to create quantum bits -- or qubits -- that are stable, meaning they are not much affected by changes in their environment. This normally needs highly nonlinear non-dissipative elements capable of functioning at very low temperatures.
In pursuit of this goal, researchers at EPFL's Laboratory of Photonics and Quantum Measurements LPQM (STI/SB), have investigated a nonlinear graphene-based...
Dental plaque and the viscous brown slime in drainpipes are two familiar examples of bacterial biofilms. Removing such bacterial depositions from surfaces is...
For the first time, scientists have succeeded in studying the strength of hydrogen bonds in a single molecule using an atomic force microscope. Researchers from the University of Basel’s Swiss Nanoscience Institute network have reported the results in the journal Science Advances.
Hydrogen is the most common element in the universe and is an integral part of almost all organic compounds. Molecules and sections of macromolecules are...
22.05.2017 | Event News
17.05.2017 | Event News
16.05.2017 | Event News
22.05.2017 | Materials Sciences
22.05.2017 | Life Sciences
22.05.2017 | Physics and Astronomy