Proteasome-mediated degradation of integral inner nuclear membrane protein emerin in fibroblasts lacking A-type lamins.

@article{Muchir2006ProteasomemediatedDO,
  title={Proteasome-mediated degradation of integral inner nuclear membrane protein emerin in fibroblasts lacking A-type lamins.},
  author={Antoine Muchir and Catherine Massart and Baziel G. M. van Engelen and Martin Lammens and Gis{\`e}le Bonne and Howard J Worman},
  journal={Biochemical and biophysical research communications},
  year={2006},
  volume={351 4},
  pages={1011-7}
}
We previously identified and characterized a homozygous LMNA nonsense mutation leading to the absence of A-type lamins in a premature neonate who died at birth. We show here that the absence of A-type lamins is due to degradation of the aberrant mRNA transcript with a premature termination codon. In cultured fibroblasts from the subject with the homozygous LMNA nonsense mutation, there was a decreased steady-state expression of the integral inner nuclear membrane proteins emerin and nesprin… CONTINUE READING

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