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Title: Disease-related misfolding of the myelin proteolipid protein
Author: Roboti, Peristera
ISNI:       0000 0004 2668 106X
Awarding Body: The University of Manchester
Current Institution: University of Manchester
Date of Award: 2008
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A wide range of mutations in the myelin integral plasma membrane proteolipid protein (PLP) are associated with dysmyelinating diseases of varying severity, and whilst missense mutations in PLP transmembrane domains cause severe disease few such mutants result in a mild phenotype. The molecular pathology of such diseases has generally been attributed to endoplasmic reticulum (ER) retention of misfolded ing in the induction of ER stress. However, the cellular mechanism(s) that control the observed phenotypic variations have not yet been elucidated. The work documented in this thesis established that the cellular fate of three distinct transmembrane missense mutants of PLP is differentially regulated by the ER quality control process upon stable inducible expression in HeLa cells.
Supervisor: Not available Sponsor: Not available
Qualification Name: Thesis (Ph.D.) Qualification Level: Doctoral
EThOS ID:  DOI: Not available