ISOLATION AND CHARACTERIZATION OF THE PORCINE PROTEOLIPID PROTEIN (PLP) GENE

The proteolipid protein (PLP) is a highly conserved major membrane pro tein in the central nervous system (CNS). A series of neurological dis orders caused by mutations in the X-chromosomal PLP gene were describe d among mammals comprising jimpy in mouse, Pelizaeus-Merzbacher Diseas e and Spastic Paraplegia type 2 in humans, shaking pup in dop, and par alytic tremor in rabbit. These disorders share common features compris ing tremor, nystagmus, ataxia, and a high degree of lethality. The X-l inked porcine congenital tremor type A III (CT AIII) shows a high degr ee of similarity on the morphologic and cellular level to these PLP-li nked disorders. Affected piglets suffer from cramps, vertical and late ral shaking of the head and rump as well as spontaneous limb movement. The axons in brain and spinal cord are only loosely enwrapped and the number of of oligodendrocytes is decreased. Due to these similarities we propose the involvement of PLP in establishment of CT AIII which h as never been investigated on the molecular level. This study describe s the isolation and partial characterization of the porcine PLP at the transcriptional and genomic level.