Characterization of amyotrophic lateral sclerosis-linked P56S mutation of vesicle-associated membrane protein-associated protein B (VAPB/ALS8).

@article{Kanekura2006CharacterizationOA,
  title={Characterization of amyotrophic lateral sclerosis-linked P56S mutation of vesicle-associated membrane protein-associated protein B (VAPB/ALS8).},
  author={Kohsuke Kanekura and Ikuo Nishimoto and Sadakazu Aiso and Masaaki Matsuoka},
  journal={The Journal of biological chemistry},
  year={2006},
  volume={281 40},
  pages={30223-33}
}
The P56S mutation in VAPB (vesicle-associated membrane protein-associated protein B) causes autosomal dominant motoneuronal diseases. Although it was reported that the P56S mutation induces localization shift of VAPB from endoplasmic reticulum (ER) to non-ER compartments, it remains unclear what the physiological function of VAPB is and how the P56S mutation in VAPB causes motoneuronal diseases. Here we demonstrate that overexpression of wild type VAPB (wt-VAPB) promotes unfolded protein… CONTINUE READING
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