Insertion of mutant proteolipid protein results in missorting of myelin proteins
نویسندگان
چکیده
منابع مشابه
Assembly of CNS Myelin in the Absence of Proteolipid Protein
Two proteolipid proteins, PLP and DM20, are the major membrane components of central nervous system (CNS) myelin. Mutations of the X-linked PLP/DM20 gene cause dysmyelination in mouse and man and result in significant mortality. Here we show that mutant mice that lack expression of a targeted PLP gene fail to exhibit the known dysmyelinated phenotype. Unable to encode PLP/DM20 or PLP-related po...
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Although proteolipid protein (PLP) and its DM20 isoform are the major membrane proteins of CNS myelin, their absence causes surprisingly few developmental defects. In comparison, missense mutations of the X-linked Plp gene cause severe dysmyelination. Previous studies have established roles for PLP/DM20 in the formation of the intraperiod line and in maintaining axonal integrity. We now show th...
متن کاملMyelin basic protein is affected by reduced synthesis of myelin proteolipid protein in the jimpy mouse.
Myelin basic proteins (MBPs) from 6-day-old, 10-day-old, 20-day-old and adult normal mouse brain were compared with those from 20-day-old jimpy (dysmyelinating mutant) mouse brain to determine the effect of reduced levels of proteolipid protein (PLP) on MBPs. Alkaline-urea-gel electrophoresis showed that 6-day-old and 10-day-old normal and jimpy MBPs lacked charge microheterogeneity, since C8 (...
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Vertebrate myelin contains two proteins that mediate compaction: protein zero (P0), an immunoglobulin gene superfamily member, or proteolipid proteins, 4-hydrophobic domain-motif proteins biogenetically unrelated to P0. The prevailing view has been that expression of P0 and proteolipid proteins is mutually exclusive; P0, which mediates myelin compaction in fish, is thought to be completely repl...
متن کاملIn Vivo Acylation of Rat Brain Myelin Proteolipid Protein*
Examination of brain myelin proteins by sodium dodecyl sulfate-gel electrophoresis followed by fluorography clearly showed that both proteolipid protein (PLP) and DM-20 were acylated 24 h after the intracerebral injection of 30-day-old rats with C3H]palmitic acid. The radioactivity associated with PLP remained after purification, re-electrophoresis, and fluorography. Most of the radioactivity a...
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ژورنال
عنوان ژورنال: Annals of Neurology
سال: 2003
ISSN: 0364-5134,1531-8249
DOI: 10.1002/ana.10762