A team of researchers headed by Douglas R. Cavener, professor and head of the Department of Biology at Penn State University, has announced important findings about the causes of three human diseases: severe, juvenile-onset diabetes; osteoporosis; and Wolcott-Rallison Syndrome, a rare condition whose sufferers exhibit a combination of diabetes, retarded growth, and skeletal abnormalities. Their work suggests promising lines of research for the therapeutic treatment of these diseases. The work will be described in an article in the August 2003 issue of the journal Endocrinology.
Three mice that are littermates (siblings of the same age). The two mice on the left (one partially hiding under the big mouse) are dwarf mice caused by the Perk mutation. They are less than half the size of the mouse on the right, which is normal.
Micro x-ray computed tomography (microCT) images of the tibia of normal and Perk mutant mice. The mutant shows thin cortical bone with deformations and gaping holes. "If it wasnt for muscle and connective tissue to hold these fragile bones together, these mice would fall apart," Cavener says.
Over several years, Caveners team has developed and investigated a particular strain of "knockout" mice that are genetically unable to produce the enzyme PERK (pancreatic endoplasmic reticulum kinase). Cavener and his team hypothesize that the PERK enzyme is a specific or global regulator of protein synthesis, which means that the knockout mice are especially useful in unraveling complex physiological and developmental processes. "We are now able to investigate what happens when a particular gene is missing, to see what functions go wrong. Then we work backwards to fill in mechanisms that link the genotype to the phenotype" or the observable outcome, Cavener says.
PERK knockout mice have a very low survival rate because the biomedical problems caused by the absence of the PERK enzyme are so severe. Fully 63 percent die during gestation or the first few days of life which can be traced to failures in the creation or delivery of specific proteins.
Barbara K. Kennedy | EurekAlert!
Scientists use nanoparticle-delivered gene therapy to inhibit blinding eye disease in rodents
08.07.2020 | Johns Hopkins Medicine
Deconstructing glioblastoma complexity reveals its pattern of development
08.07.2020 | McGill University
New insight into the spin behavior in an exotic state of matter puts us closer to next-generation spintronic devices
Aside from the deep understanding of the natural world that quantum physics theory offers, scientists worldwide are working tirelessly to bring forth a...
Kiel physics team observed extremely fast electronic changes in real time in a special material class
In physics, they are currently the subject of intensive research; in electronics, they could enable completely new functions. So-called topological materials...
Solar cells based on perovskite compounds could soon make electricity generation from sunlight even more efficient and cheaper. The laboratory efficiency of these perovskite solar cells already exceeds that of the well-known silicon solar cells. An international team led by Stefan Weber from the Max Planck Institute for Polymer Research (MPI-P) in Mainz has found microscopic structures in perovskite crystals that can guide the charge transport in the solar cell. Clever alignment of these "electron highways" could make perovskite solar cells even more powerful.
Solar cells convert sunlight into electricity. During this process, the electrons of the material inside the cell absorb the energy of the light....
Empa researchers have succeeded in applying aerogels to microelectronics: Aerogels based on cellulose nanofibers can effectively shield electromagnetic radiation over a wide frequency range – and they are unrivalled in terms of weight.
Electric motors and electronic devices generate electromagnetic fields that sometimes have to be shielded in order not to affect neighboring electronic...
A promising operating mode for the plasma of a future power plant has been developed at the ASDEX Upgrade fusion device at Max Planck Institute for Plasma...
07.07.2020 | Event News
02.07.2020 | Event News
19.05.2020 | Event News
10.07.2020 | Materials Sciences
10.07.2020 | Life Sciences
10.07.2020 | Materials Sciences