pH-dependent stability of neuroserpin is mediated by histidines 119 and 138; implications for the control of beta-sheet A and polymerization.

@article{Belorgey2010pHdependentSO,
  title={pH-dependent stability of neuroserpin is mediated by histidines 119 and 138; implications for the control of beta-sheet A and polymerization.},
  author={Didier Belorgey and Peter H{\"a}ggl{\"o}f and Maki Onda and David A. Lomas},
  journal={Protein science : a publication of the Protein Society},
  year={2010},
  volume={19 2},
  pages={220-8}
}
Neuroserpin is a member of the serpin superfamily. Point mutations in the neuroserpin gene underlie the autosomal dominant dementia, familial encephalopathy with neuroserpin inclusion bodies. This is characterized by the retention of ordered polymers of neuroserpin within the endoplasmic reticulum of neurons. pH has been shown to affect the propensity of several serpins to form polymers. In particular, low pH favors the formation of polymers of both alpha(1)-antitrypsin and antithrombin. We… CONTINUE READING
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