The human disease, known as Hemophagocytic Lymphohistiocytosis, or HLH, is a rare inflammatory disease that kills between 50 percent and 90 percent of its victims, said Diane Brown, lead author of the study. HLH treatment may require bone marrow transplantation, a drastic therapy with life-long consequences, according to Brown, adjoint curator at the University of Colorado Museum of Natural History.
The disease, in which the immune system becomes hyperactivated, occurs both in an inherited form, known as primary HLH, and in people with no known genetic defect, known as secondary HLH. Both forms are usually triggered by infections. The genetic form of HLH most often strikes infants and very young children.
A paper on the subject is being published Feb. 26 in PLoS One, a journal of the Public Library of Science. Study co-authors, all from CU-Boulder's molecular, cellular and developmental biology department, included Assistant Professor Corrella Detweiler, postdoctoral fellows Melissa McCoy and Carolina Pilonieta, and former master's student Rebecca Nix, now at Supergen in Salt Lake City. The study was funded by the National Institutes of Health and the American Recovery and Relief Act.
Salmonella is a well-known food contaminant, causing a variety of gastrointestinal tract symptoms in humans. While mice infected with salmonella are spared the cramping and diarrhea that humans develop, they were shown to develop a disease syndrome comparable to human secondary HLH, said Brown.
The CU-Boulder research team found that mice infected with salmonella developed fever, enlarged spleens, anemia, reduced numbers of platelets, dangerously high blood levels of an iron-storage protein, and neurological signs. In addition, specialized white blood cells known as hemophagocytic macrophages accumulated in the organs of the body, including bone marrow.
The team previously showed that salmonella-infected mice developed hemophagocytic macrophages, which ingest other white and red blood cells. "These earlier findings helped lead us down the current research path," said Detweiler.
"One part of this study is to try to use our research to understand how anemia develops in these infected mice, which might help us understand how symptoms of HLH develop," said Detweiler. The syndrome can be difficult to recognize and diagnose, she added.
"The availability of this animal model for HLH will help to advance the research and understanding of the underlying mechanisms of this immune system disorder," said Detweiler. "It also should provide a means to test new therapies for HLH."For more information about MCDB visit http://mcdb.colorado.edu/.
Diane Brown | EurekAlert!
Further reports about: > Hemophagocytic Lymphohistiocytosis > PLoS One > blood cell > blood cells > bone marrow > gastrointestinal tract symptoms > hemophagocytic macrophages > human inflammatory disease > immune system > inflammatory disease > iron-storage protein > salmonella-infected mice > white blood cell
Amputees can learn to control a robotic arm with their minds
28.11.2017 | University of Chicago Medical Center
The importance of biodiversity in forests could increase due to climate change
17.11.2017 | Deutsches Zentrum für integrative Biodiversitätsforschung (iDiv) Halle-Jena-Leipzig
Tiny pores at a cell's entryway act as miniature bouncers, letting in some electrically charged atoms--ions--but blocking others. Operating as exquisitely sensitive filters, these "ion channels" play a critical role in biological functions such as muscle contraction and the firing of brain cells.
To rapidly transport the right ions through the cell membrane, the tiny channels rely on a complex interplay between the ions and surrounding molecules,...
The miniaturization of the current technology of storage media is hindered by fundamental limits of quantum mechanics. A new approach consists in using so-called spin-crossover molecules as the smallest possible storage unit. Similar to normal hard drives, these special molecules can save information via their magnetic state. A research team from Kiel University has now managed to successfully place a new class of spin-crossover molecules onto a surface and to improve the molecule’s storage capacity. The storage density of conventional hard drives could therefore theoretically be increased by more than one hundred fold. The study has been published in the scientific journal Nano Letters.
Over the past few years, the building blocks of storage media have gotten ever smaller. But further miniaturization of the current technology is hindered by...
With innovative experiments, researchers at the Helmholtz-Zentrums Geesthacht and the Technical University Hamburg unravel why tiny metallic structures are extremely strong
Light-weight and simultaneously strong – porous metallic nanomaterials promise interesting applications as, for instance, for future aeroplanes with enhanced...
An interdisciplinary group of researchers interfaced individual bacteria with a computer to build a hybrid bio-digital circuit - Study published in Nature Communications
Scientists at the Institute of Science and Technology Austria (IST Austria) have managed to control the behavior of individual bacteria by connecting them to a...
Physicists in the Laboratory for Attosecond Physics (run jointly by LMU Munich and the Max Planck Institute for Quantum Optics) have developed an attosecond electron microscope that allows them to visualize the dispersion of light in time and space, and observe the motions of electrons in atoms.
The most basic of all physical interactions in nature is that between light and matter. This interaction takes place in attosecond times (i.e. billionths of a...
11.12.2017 | Event News
08.12.2017 | Event News
07.12.2017 | Event News
11.12.2017 | Physics and Astronomy
11.12.2017 | Earth Sciences
11.12.2017 | Information Technology