Genetic dissection of oligodendroglial and neuronal<i>Plp1</i>function in a novel mouse model of spastic paraplegia type 2

Abstract excerpt

Proteolipid protein (PLP) is the most abundant integral membrane protein in compact central nervous system myelin, and null mutations of the PLP1 gene cause spastic paraplegia type 2 (SPG2). SPG2 patients and PLP-deficient mice exhibit only moderate abnormalities of myelin but progressive degeneration of long axons. Since Plp1 gene products are detected in a subset of neurons it has been suggested that the loss...

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