Proteolipid Protein Is Required for Transport of Sirtuin 2 into CNS Myelin
نویسندگان
چکیده
منابع مشابه
Proteolipid protein is required for transport of sirtuin 2 into CNS myelin.
Mice lacking the expression of proteolipid protein (PLP)/DM20 in oligodendrocytes provide a genuine model for spastic paraplegia (SPG-2). Their axons are well myelinated but exhibit impaired axonal transport and progressive degeneration, which is difficult to attribute to the absence of a single myelin protein. We hypothesized that secondary molecular changes in PLP(null) myelin contribute to t...
متن کاملAssembly 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...
متن کاملAdhesive properties of proteolipid protein are responsible for the compaction of CNS myelin sheaths.
We have studied the molecular function of proteolipid protein (PLP), the main integral membrane protein of CNS myelin, by generating mice lacking PLP expression. Here, we demonstrate that these PLP-minus mice show no pleiotropism as mice carrying point mutations within the PLP gene. The expression of other myelin genes (myelin basic protein, MBP; myelin associated glycoprotein, MAG; UDP-galacto...
متن کاملTransport of the major myelin proteolipid protein is directed by VAMP3 and VAMP7.
CNS myelination by oligodendrocytes requires directed transport of myelin membrane components and a timely and spatially controlled membrane expansion. In this study, we show the functional involvement of the R-soluble N-ethylmaleimide-sensitive factor attachment protein receptor (R-SNARE) proteins VAMP3/cellubrevin and VAMP7/TI-VAMP in myelin membrane trafficking. VAMP3 and VAMP7 colocalize wi...
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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ژورنال
عنوان ژورنال: Journal of Neuroscience
سال: 2007
ISSN: 0270-6474,1529-2401
DOI: 10.1523/jneurosci.1254-07.2007