Nucleocytoplasmic transport in cells with progerin-induced defective nuclear lamina
Copyright policy )Nucleocytoplasmic transport in cells with progerin-induced defective nuclear lamina
Recent data indicate that nuclear lamina (NL) plays a relevant role in many fundamental cellular functions. The peculiar role of NL in cells is dramatically demonstrated by the Hutchinson-Gilford progeria syndrome (HGPS), an inherited laminopathy that causes premature, rapid aging shortly after birth. In HGPS, a mutant form of Lamin A (progeria) leads to a dysmorphic NL structure, but how this perturbation is transduced into cellular changes is still largely unknown. Owing to the close structural relationship between NL and the Nuclear Pore Complex (NPC), in this work we test whether HGPS affects passive and active nucleo-cytoplasmic shuttling of cargoes by means of an established model based of fluorescence recovery after photobleaching. Our findings clearly demonstrate that dysmorphic NL is decoupled from the dynamic characteristics of passive and active transport towards and from the nucleus, as well as from the binding affinity of transport protein mediators.
- Scuola Normale Superiore Italy
- National Research Council Italy
- National Research Council Romania
- Italian Institute of Technology Italy
- Nest Labs United States
Nuclear lamin, Nuclear export signal (NES), Nuclear localization signal (NLS), Nucleocytoplasmic transport, Nuclear Lamina, Active Transport, Cell Nucleus, Cell Line, Tumor, Fluorescence Recovery After Photobleaching, FRAP, Humans, Lamin Type A, Microscopy, Fluorescence, Models, Biological, Nuclear export signal (NES), Nuclear lamin, Nuclear Lamina, Nuclear localization signal (NLS), Nucleocytoplasmic transport, Progeria, Protein Precursors, Recombinant Fusion Proteins, Recombinant Fusion Proteins, Active Transport, Cell Nucleus, Lamin Type A, Models, Biological, Progeria, Microscopy, Fluorescence, Cell Line, Tumor, FRAP, Humans, Protein Precursors, Fluorescence Recovery After Photobleaching
Nuclear lamin, Nuclear export signal (NES), Nuclear localization signal (NLS), Nucleocytoplasmic transport, Nuclear Lamina, Active Transport, Cell Nucleus, Cell Line, Tumor, Fluorescence Recovery After Photobleaching, FRAP, Humans, Lamin Type A, Microscopy, Fluorescence, Models, Biological, Nuclear export signal (NES), Nuclear lamin, Nuclear Lamina, Nuclear localization signal (NLS), Nucleocytoplasmic transport, Progeria, Protein Precursors, Recombinant Fusion Proteins, Recombinant Fusion Proteins, Active Transport, Cell Nucleus, Lamin Type A, Models, Biological, Progeria, Microscopy, Fluorescence, Cell Line, Tumor, FRAP, Humans, Protein Precursors, Fluorescence Recovery After Photobleaching
selected citations These citations are derived from selected sources.This is an alternative to the "Influence" indicator, which also reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).12 popularity This indicator reflects the "current" impact/attention (the "hype") of an article in the research community at large, based on the underlying citation network.Top 10% influence This indicator reflects the overall/total impact of an article in the research community at large, based on the underlying citation network (diachronically).Average impulse This indicator reflects the initial momentum of an article directly after its publication, based on the underlying citation network.Top 10%
Citations provided by BIP!Popularity provided by BIP!