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The posttranslational processing of prelamin A and disease Davies BS; Fong LG; Yang SH; Coffinier C; Young SGAnnu Rev Genomics Hum Genet 2009[]; 10 (ä): 153-74Human geneticists have shown that some progeroid syndromes are caused by mutations that interfere with the conversion of farnesyl-prelamin A to mature lamin A. For example, Hutchinson-Gilford progeria syndrome is caused by LMNA mutations that lead to the accumulation of a farnesylated version of prelamin A. In this review, we discuss the posttranslational modifications of prelamin A and their relevance to the pathogenesis and treatment of progeroid syndromes.|*Protein Processing, Post-Translational[MESH]|Animals[MESH]|Disease Models, Animal[MESH]|Enzyme Inhibitors/therapeutic use[MESH]|Farnesyltranstransferase/antagonists & inhibitors[MESH]|Humans[MESH]|Infant, Newborn[MESH]|Lamin Type A[MESH]|Nuclear Proteins/genetics/*metabolism[MESH]|Progeria/drug therapy/genetics/*metabolism[MESH]|Protein Precursors/genetics/*metabolism[MESH] |
