381 The role of leucine-rich PPR motif-containing protein (LRPPRC) in myelin lipid metabolism
OBJECTIVES/GOALS: Leigh Syndrome, French Canadian-Type (LSFC) is a neurometabolic disorder caused by mutation of mitochondria-related gene, LRPPRC. White matter lesions and demyelination in central nervous system are common in LSFC. LRPPRC is enriched in
Bridgitte Palacios +2 more
doaj +1 more source
Reduced experimental autoimmune encephalomyelitis after intranasal and oral administration of recombinant lactobacilli expressing myelin antigen [PDF]
Oral administration of autoantigens is a safe and convenient way to induce peripheral T-cell tolerance in autoimmune diseases like multiple sclerosis (MS).
Boersma, W.G. (Wim) +8 more
core +1 more source
Thiopalmitoylation of Myelin Proteolipid Protein Epitopes Enhances Immunogenicity and Encephalitogenicity [PDF]
Abstract Proteolipid protein (PLP) is the most abundant protein of CNS myelin, and is posttranslationally acylated by covalent attachment of long chain fatty acids to cysteine residues via a thioester linkage. Two of the acylation sites are within epitopes of PLP that are encephalitogenic in SJL/J mice (PLP104–117 and PLP139–151) and ...
Greer, J. M. +3 more
openaire +3 more sources
Kir4.1 Potassium Channel Subunit Is Crucial for Oligodendrocyte Development and In Vivo Myelination [PDF]
To understand the cellular and in vivo functions of specific K^+ channels in glia, we have studied mice with a null mutation in the weakly inwardly rectifying K^+ channel subunit Kir4.1.
Jacobs, Russell E. +4 more
core +1 more source
The Microbiota Shapes Central Nervous System Myelination in Early Life
Gut microbiota shapes brain development by regulating myelination and glial cell maturation in early life. Using germ‐free (GF) mice and zebrafish, this study reveals sex‐ and age‐dependent effects on myelin growth, integrity, and related gene expression.
Caoimhe M. K. Lynch +13 more
wiley +1 more source
Distinctive Structural and Molecular Features of Myelinated Inhibitory Axons in Human Neocortex. [PDF]
Numerous types of inhibitory neurons sculpt the performance of human neocortical circuits, with each type exhibiting a constellation of subcellular phenotypic features in support of its specialized functions.
Chang, Edward F +9 more
core +1 more source
Demyelination and Early Remyelination in Experimental Allergic Encephalomyelitis Passively Transferred With Myelin Basic Protein-Sensitized Lymphocytes in the Lewis Rat [PDF]
Histological studies were performed on Lewis rats with experimental allergic encephalomyelitis (EAE) passively transferred by myelin basic protein (MBP)-sensitized syngeneic spleen cells in order to determine the relationship between demyelination and ...
Ben-Nun +49 more
core +1 more source
Autoacylation of myelin proteolipid protein with acyl coenzyme A.
Rat brain myelin proteolipid protein (PLP) is known to contain long chain, covalently bound fatty acids. In the course of characterizing the mechanism of acylation, we found that the isolated PLP, in the absence of any membrane fraction, was esterified ...
O. Bizzozero, J. McGarry, M. Lees
semanticscholar +1 more source
Myelin pathology: Involvement of molecular chaperones and the promise of chaperonotherapy [PDF]
The process of axon myelination involves various proteins including molecular chaperones. Myelin alteration is a common feature in neurological diseases due to structural and functional abnormalities of one or more myelin proteins.
Cappello F. +4 more
core +1 more source
CDC42‐Effector Proteins Regulate Higher Order Structure of Septins Required for CNS Myelin Integrity
CDC42‐effector proteins 1/2 are present in CNS myelin. They facilitate the higher order structure of myelin septin filaments. Their loss impairs septin‐dependent scaffolding of myelin. Myelin outfoldings do not cause secondary neuropathology per se. ABSTRACT The regular structure of CNS myelin requires specialized structural proteins, including septin ...
Sophie Hümmert +14 more
wiley +1 more source

