An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria
نویسندگان
چکیده
منابع مشابه
An accumulation of non-farnesylated prelamin A causes cardiomyopathy but not progeria.
Lamin A is formed from prelamin A by four post-translational processing steps-farnesylation, release of the last three amino acids of the protein, methylation of the farnesylcysteine and the endoproteolytic release of the C-terminal 15 amino acids (including the farnesylcysteine methyl ester). When the final processing step does not occur, a farnesylated and methylated prelamin A accumulates in...
متن کاملAutophagic degradation of farnesylated prelamin A as a therapeutic approach to lamin-linked progeria
Farnesylated prelamin A is a processing intermediate produced in the lamin A maturation pathway. Accumulation of a truncated farnesylated prelamin A form, called progerin, is a hallmark of the severe premature ageing syndrome, Hutchinson-Gilford progeria. Progerin elicits toxic effects in cells, leading to chromatin damage and cellular senescence and ultimately causes skin and endothelial defec...
متن کاملErratum - Autophagic Degradation of Farnesylated Prelamin a as a Therapeutic Approach to Lamin-Linked Progeria
V. Cenni,1 C. Capanni,1 M. Columbaro,2 M. Ortolani,1 M.R. D’Apice,3 G. Novelli,4 M. Fini,5 S. Marmiroli,6 E. Scarano,7 N.M. Maraldi,2 S. Squarzoni,1 S. Prencipe,5 G. Lattanzi1 1National Research Council of Italy, Institute for Molecular Genetics, IGM-CNR, Unit of Bologna c/o IOR, Bologna 2Laboratory of Musculoskeletal Cell Biology, Rizzoli Orthopedic Institute, Bologna 3Department of Biopatholo...
متن کاملAccumulation of prelamin A compromises NF-κB-regulated B-lymphopoiesis in a progeria mouse model
BACKGROUND Alteration in the immune system is one of the most profound aspects of aging. Progressive changes in the number of B lymphocyte progenitors during aging have been reported but the underlying mechanisms are still elusive. A heterozygous G608G mutation in the LMNA gene leads to a deletion of 50 amino acids in lamin A protein, termed progerin, and is the predominant cause of Hutchinson-...
متن کاملPrelamin A causes progeria through cell-extrinsic mechanisms and prevents cancer invasion
Defining the relationship between ageing and cancer is a crucial but challenging task. Mice deficient in Zmpste24, a metalloproteinase mutated in human progeria and involved in nuclear prelamin A maturation, recapitulate multiple features of ageing. However, their short lifespan and serious cell-intrinsic and cell-extrinsic alterations restrict the application and interpretation of carcinogenes...
متن کاملذخیره در منابع من
با ذخیره ی این منبع در منابع من، دسترسی به آن را برای استفاده های بعدی آسان تر کنید
ژورنال
عنوان ژورنال: Human Molecular Genetics
سال: 2010
ISSN: 0964-6906,1460-2083
DOI: 10.1093/hmg/ddq158