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ESCRT-III Dysfunction Causes Autophagosome Accumulation and Neurodegeneration
Zmpste24 deficiency in mice causes spontaneous bone fractures, muscle weakness, and a prelamin A processing defect
- M. Bergo, Bryant J. Gavino, S. Young
- Biology, MedicineProceedings of the National Academy of Sciences…
- 16 September 2002
Zmpste24 is an integral membrane metalloproteinase of the endoplasmic reticulum. Biochemical studies of tissues from Zmpste24-deficient mice (Zmpste24−/−) have indicated a role for Zmpste24 in the…
Glycosylphosphatidylinositol-anchored high-density lipoprotein-binding protein 1 plays a critical role in the lipolytic processing of chylomicrons.
Analysis of the role of microsomal triglyceride transfer protein in the liver of tissue-specific knockout mice.
It is concluded that MTP is essential for transferring the bulk of triglycerides into the lumen of the ER for VLDL assembly and is required for the secretion of apo B-100 from the liver.
BayGenomics: a resource of insertional mutations in mouse embryonic stem cells
The BayGenomics gene-trap resource (http://baygenomics.ucsf.edu) provides researchers with access to thousands of mouse embryonic stem (ES) cell lines harboring characterized insertional mutations in…
Lamins A and C but Not Lamin B1 Regulate Nuclear Mechanics*
- J. Lammerding, L. Fong, Richard T. Lee
- Biology, EngineeringJournal of Biological Chemistry
- 1 September 2006
It is indicated that lamins A and C are important contributors to the mechanical stiffness of nuclei, whereas lamin B1 contributes to nuclear integrity but not stiffness.
Lamin B1 is required for mouse development and nuclear integrity.
- L. Vergnes, M. Péterfy, M. Bergo, S. Young, K. Reue
- BiologyProceedings of the National Academy of Sciences…
- 13 July 2004
These mutant mice and cell lines derived from them will be useful models for studying the role of the nuclear lamina in various cellular processes and provide evidence for a broad and nonredundant function of lamin B1 in mammalian development.
The Knockout Mouse Project
It is time to harness new technologies and efficiencies of production to mount a high-throughput international effort to produce and phenotype knockouts for all mouse genes, and place these resources into the public domain.
Phenotypes of apolipoprotein B and apolipoprotein E after liver transplantation.
In all 29 patients that were studied, the postoperative serum apoE phenotype of the recipient, as assessed by isoelectric focusing, converted virtually completely to that of the donor, providing evidence that greater than 90% of the apo E in the plasma is synthesized by the liver, and indicating that most of the ApoE in CSF cannot be derived from the plasma pool and therefore must be synthesized locally.
Blocking protein farnesyltransferase improves nuclear blebbing in mouse fibroblasts with a targeted Hutchinson-Gilford progeria syndrome mutation.
A gene-targeted mouse model of HGPS was created, genetically identical primary mouse embryonic fibroblasts were generated, and the effect of a farnesyltransferase inhibitor on nuclear blebbing was examined, suggesting a possible treatment strategy for HGPS.