title: Progeria Research Day at Brunel University.
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.
authors: Centre of Cell & Chromosome Biology, Brunel Institute of Ageing Studies, Brunel University, London, UK. Joanna.bridger@brunel.ac.uk
authors: Joanna M
authors: JM
authors: Bridger
authors: Christopher H
authors: CH
authors: Eskiw
authors: Evgeny M
authors: EM
authors: Makarov
authors: David
authors: D
authors: Tree
authors: Ian R
authors: IR
authors: Kill
mesh_terms: D035843:Biomedical Research; D004739:England; D006801:Humans; D034904:Lamin Type A; D009154:Mutation; D009687:Nuclear Proteins / Q000235:genetics / Q000378:metabolism*; D011371:Progeria / Q000235:genetics / Q000378:metabolism*; D011498:Protein Precursors / Q000235:genetics / Q000378:metabolism*; D014495:Universities
chemical_list: D034904:Lamin Type A; D009687:Nuclear Proteins; D011498:Protein Precursors; C090764:prelamin A