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Genetic Background Influences UPR but not PLP Processing in the rumpshaker Model of PMD/SPG2
Mutations of the proteolipid protein gene (PLP1) cause Pelizaeus-Merzbacher disease (PMD) and Spastic paraplegia type 2 (SPG2). The rumpshaker mutation is associated with mild forms of PMD or SPG2 in man and the ...