1. Progeria Research Day at Brunel University.
- Author
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Bridger JM, Eskiw CH, Makarov EM, Tree D, and Kill IR
- Subjects
- England, Humans, Lamin Type A, Nuclear Proteins genetics, Progeria genetics, Protein Precursors genetics, Biomedical Research, Mutation, Nuclear Proteins metabolism, Progeria metabolism, Protein Precursors metabolism, Universities
- Abstract
Hutchinson-Gilford Progeria Syndrome (HGPS) is a severe premature aging syndrome that affects children. These children display characteristics associated with normal aging and die young usually from cardiovascular problems or stroke. Classical HGPS is caused by mutations in the gene encoding the nuclear structural protein lamin A. This mutation leads to a novel version of lamin A that retains a farnesyl group from its processing. This protein is called Progerin and is toxic to cellular function. Pre-lamin A is an immature version of lamin A and also has a farnesylation modification, which is cleaved in the maturation process to create lamin A.
- Published
- 2011
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