| 1. |
|
|
| 2. |
|
|
| 3. |
|
|
| 4. |
|
|
| 5. |
|
|
| 6. |
|
|
| 7. |
|
|
| 8. |
|
|
| 9. |
- Fong, L. G., et al.
(author)
-
Heterozygosity for Lmna deficiency eliminates the progeria-like phenotypes in Zmpste24-deficient mice
- 2004
-
In: Proc Natl Acad Sci U S A. ; 101:52, s. 18111-18116
-
Journal article (peer-reviewed)abstract
- Zmpste24 is a metalloproteinase required for the processing of prelamin A to lamin A, a structural component of the nuclear lamina. Zmpste24 deficiency results in the accumulation of prelamin A within cells, a complete loss of mature lamin A, and misshapen nuclear envelopes. Zmpste24-deficient (Zmpste24(-/-)) mice exhibit retarded growth, alopecia, micrognathia, dental abnormalities, osteolytic lesions in bones, and osteoporosis, which are phenotypes shared with Hutchinson-Gilford progeria syndrome, a human disease caused by the synthesis of a mutant prelamin A that cannot undergo processing to lamin A. Zmpste24(-/-) mice also develop muscle weakness. We hypothesized that prelamin A might be toxic and that its accumulation in Zmpste24(-/-) mice is responsible for all of the disease phenotypes. We further hypothesized that Zmpste24(-/-) mice with half-normal levels of prelamin A (Zmpste24(-/-) mice with one Lmna knockout allele) would be subjected to less toxicity and be protected from disease. Thus, we bred and analyzed Zmpste24(-/-)Lmna(+/-) mice. As expected, prelamin A levels in Zmpste24(-/-)Lmna(+/-) cells were significantly reduced. Zmpste24(-/-)Lmna(+/-) mice were entirely normal, lacking all disease phenotypes, and misshapen nuclei were less frequent in Zmpste24(-/-)Lmna(+/-) cells than in Zmpste24(-/-) cells. These data suggest that prelamin A is toxic and that reducing its levels by as little as 50% provides striking protection from disease.
|
|
| 10. |
|
|
| 11. |
|
|
| 12. |
- Graff, C, et al.
(author)
-
Mitochondrial diseases
- 2002
-
In: Best practice & research. Clinical obstetrics & gynaecology. - : Elsevier BV. - 1521-6934. ; 16:5, s. 715-728
-
Journal article (peer-reviewed)
|
|
| 13. |
|
|
| 14. |
|
|
| 15. |
|
|
| 16. |
- Moore, P. R., et al.
(author)
-
Virtual engineering : an integrated approach to agile manufacturing machinery design and control
- 2003
-
In: Mechatronics (Oxford). - : BioMed Central Ltd.. - 0957-4158 .- 1873-4006. ; 13:10, s. 1105-1121
-
Journal article (peer-reviewed)abstract
- A virtual manufacturing approach for designing, programming, testing, verifying and deploying control systems for agile modular manufacturing machinery are proposed in this paper. It introduces the concepts, operations, mechanisms and implementation techniques for integrating simulation environments and distributed control system environments so that the control logic programs that have been programmed and verified in the virtual environment can be seamlessly transferred to the distributed control system environment for controlling the real devices. The approach looks to exploit simulation in a much wider range of applications with great advantages in the design and development of manufacturing machine systems. In particular, it facilitates the verification of the runtime support applications using the simulation model before they are applied to the real system. Mechanisms that allow runtime data to be collected during operation of the real machinery to calibrate the simulation models are also proposed. The system implemented delivers a powerful set of software tools for realising agile modular manufacturing systems.
|
|
| 17. |
|
|
| 18. |
|
|
| 19. |
|
|
| 20. |
|
|
| 21. |
|
|
| 22. |
|
|
| 23. |
|
|
| 24. |
- Trifunovic, A, et al.
(author)
-
Creation of mtDNA mutator mice
- 2004
-
In: BIOCHIMICA ET BIOPHYSICA ACTA-BIOENERGETICS. - 0005-2728. ; 1657, s. 21-21
-
Conference paper (other academic/artistic)
|
|
| 25. |
|
|
