Glomerular epithelial cell injury associated with mutant alpha-actinin-4.

  title={Glomerular epithelial cell injury associated with mutant alpha-actinin-4.},
  author={Andrey V Cybulsky and Tomoko Takano and Joan Papillon and Krikor Bijian and Julie Guillemette and Chris R J Kennedy},
  journal={American journal of physiology. Renal physiology},
  volume={297 4},
Focal segmental glomerulosclerosis (FSGS) may be associated with glomerular epithelial cell (GEC; podocyte) apoptosis due to acquired injury or mutations in alpha-actinin-4. This study addresses how FSGS-associated mutant alpha-actinin-4 may induce GEC injury, focusing on endoplasmic reticulum (ER) stress and metabolism of mutant alpha-actinin-4 via the ubiquitin-proteasome system. In a model of experimental FSGS induced by expression of an alpha-actinin-4 K256E transgene in podocytes, we show… CONTINUE READING
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