A-type lamins and Hutchinson-Gilford progeria syndrome: pathogenesis and therapy.

@article{Gonzlez2011AtypeLA,
  title={A-type lamins and Hutchinson-Gilford progeria syndrome: pathogenesis and therapy.},
  author={Jos{\'e} Mar{\'i}a Gonz{\'a}lez Gonz{\'a}lez and Davinia Pla and Dolores P{\'e}rez-Sala and Vicente Andr{\'e}s},
  journal={Frontiers in bioscience},
  year={2011},
  volume={3},
  pages={1133-46}
}
Lamin A and lamin C (A-type lamins, both encoded by the LMNA gene) are major components of the mammalian nuclear lamina, a complex proteinaceous structure that acts as a scaffold for protein complexes that regulate nuclear structure and function. Abnormal accumulation of farnesylated-progerin, a mutant form of prelamin A, plays a key role in the pathogenesis of the Hutchinson-Gilford progeria syndrome (HGPS), a devastating disorder that causes the death of affected children at an average age of… CONTINUE READING

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