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Pelizaeus–Merzbacher Disease (PMD) is an inherited leukodystrophy affecting the central nervous system (CNS)—a rare disorder that especially concerns males. Its estimated prevalence is 1.45–1.9 per 100,000 individuals in the general population.
Guy Khalaf +5 more
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Overview of myelin, major myelin lipids, and myelin-associated proteins
Myelin is a modified cell membrane that forms a multilayer sheath around the axon. It retains the main characteristics of biological membranes, such as lipid bilayer, but differs from them in several important respects.
Alexander Kister, Ilya Kister
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Autophagic degradation of CNS myelin maintains axon integrity
(Macro)autophagy is a major lysosome-dependent degradation mechanism which engulfs, removes and recycles unwanted cytoplasmic material, including damaged organelles and toxic protein aggregates.
Niki Ktena +8 more
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Identification of potential functional peptides involved in demyelinating injury in the central nervous system [PDF]
Multiple sclerosis (MS) is a chronic inflammatory neurologic disease characterized by the demyelinating injury of the central nervous system (CNS).
Xiaohua Dong +6 more
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Pelizaeus-Merzbacher Disease: A Case Report
Pelizaeus-Merzbacher Disease (PMD), as a rare genetically x-linked leukodystrophy, is a disorder of proteolipid protein expression in myelin formation. This disorder is clinically presented by neurodevelopmental delay and abnormal pendular eye movements.
Ghazaleh Jamalipour Soufi +1 more
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Interaction of PLP with GFP-MAL2 in the human oligodendroglial cell line HOG. [PDF]
The velocity of the nerve impulse conduction of vertebrates relies on the myelin sheath, an electrically insulating layer that surrounds axons in both the central and peripheral nervous systems, enabling saltatory conduction of the action potential ...
Raquel Bello-Morales +6 more
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Metabolic Instability of Myelin Protein and Proteolipid Fractions [PDF]
Turnover of protein components of myelin in vivo was shown to be comparable to that of other brain proteins. Myelin was purified, to remove soluble contamination, by water shock (fraction M2) and centrifugation on sucrose and CsCl gradients. These methods gave two fractions, one of which consisted of characteristic myelin membranes free of visible ...
B, D'Monte, P, Mela, N, Marks
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The levels of brain-derived neurotrophic factor (BDNF) in the corpus callosum have previously been shown to have a critical impact on oligodendrocyte (OLG) lineage cells during cuprizone-elicited demyelination. In particular, BDNF+/– mice exhibit greater
Yangyang Huang +6 more
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A Journey to the Conformational Analysis of T-Cell Epitope Peptides Involved in Multiple Sclerosis
Multiple sclerosis (MS) is a serious central nervous system (CNS) disease responsible for disability problems and deterioration of the quality of life. Several approaches have been applied to medications entering the market to treat this disease. However,
Catherine Koukoulitsa +4 more
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A Myelin Proteolipid Protein-LacZ Fusion Protein Is Developmentally Regulated and Targeted to the Myelin Membrane in Transgenic Mice [PDF]
Transgenic mice were generated with a fusion gene carrying a portion of the murine myelin proteolipid protein (PLP) gene, including the first intron, fused to the E. coli LacZ gene.
Duchala, Cynthia S. +3 more
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