[HTML][HTML] Mutants of neuroserpin that cause dementia accumulate as polymers within the endoplasmic reticulum
E Miranda, K Römisch, DA Lomas - Journal of Biological Chemistry, 2004 - ASBMB
The dementia familial encephalopathy with neuroserpin inclusion bodies (FENIB) is caused
by the accumulation of mutant neuroserpin within neurons (Davis, RL, Shrimpton, AE,
Holohan, PD, Bradshaw, C., Feiglin, D., Sonderegger, P., Kinter, J., Becker, LM, Lacbawan,
F., Krasnewich, D., Muenke, M., Lawrence, DA, Yerby, MS, Shaw, C.-M., Gooptu, B., Elliott,
PR, Finch, JT, Carrell, RW, and Lomas, DA (1999) Nature 401, 376–379), but little is known
about the trafficking of wild type and mutant neuroserpins. We have established a cell model …
by the accumulation of mutant neuroserpin within neurons (Davis, RL, Shrimpton, AE,
Holohan, PD, Bradshaw, C., Feiglin, D., Sonderegger, P., Kinter, J., Becker, LM, Lacbawan,
F., Krasnewich, D., Muenke, M., Lawrence, DA, Yerby, MS, Shaw, C.-M., Gooptu, B., Elliott,
PR, Finch, JT, Carrell, RW, and Lomas, DA (1999) Nature 401, 376–379), but little is known
about the trafficking of wild type and mutant neuroserpins. We have established a cell model …