Results 51 to 60 of about 6,136 (180)

Unusual Presentation of Pelizaeus-Merzbacher Disease: Female Patient with Deletion of the Proteolipid Protein 1 Gene

Case Reports in Genetics, 2015
Pelizaeus-Merzbacher disease (PMD) is neurodegenerative leukodystrophy caused by dysfunction of the proteolipid protein 1 (PLP1) gene on Xq22, which codes for an essential myelin protein.
Teva Brender, Donna Wallerstein, John Sum, Robert Wallerstein   +3 more
doaj   +1 more source

Making Myelin Basic Protein -from mRNA transport to localized translation

Frontiers in Cellular Neuroscience, 2013
In the central nervous system (CNS) of most vertebrates, oligodendrocytes enwrap neuronal axons with extensions of their plasma membrane to form the myelin sheath. Several proteins are characteristically found in myelin of which Myelin Basic Protein (MBP)
Christina eMüller, Nina Marita Bauer, Isabelle eSchäfer, Robin eWhite   +3 more
doaj   +1 more source

HLA Class II Genotype Does Not Affect the Myelin Responsiveness of Multiple Sclerosis Patients

Cells, 2020
Background: When aiming to restore myelin tolerance using antigen-specific treatment approaches in MS, the wide variety of myelin-derived antigens towards which immune responses are targeted in multiple sclerosis (MS) patients needs to be taken into ...
Judith Derdelinckx, Irene Nkansah, Naomi Ooms, Laura Van Bruggen, Marie-Paule Emonds, Liesbeth Daniëls, Tatjana Reynders, Barbara Willekens, Patrick Cras, Zwi N. Berneman, Nathalie Cools   +10 more
doaj   +1 more source

Oral administration of Lactococcus lactis expressing synthetic genes of myelin antigens in decreasing experimental autoimmune encephalomyelitis in rats [PDF]

, 2015
Background: Multiple sclerosis is a human autoimmunological disease that causes neurodegeneration. One of the potential ways to stop its development is induction of oral tolerance, whose effect lies in decreasing immune response to the fed antigen.
Bardowski, Jacek K., Kasarello, Kaja, Kwiatkowska-Patzer, Barbara, Lipkowski, Andrzej, Szczepankowska, Agnieszka K.   +4 more
core   +1 more source

Reduced Oligodendrocyte Density and Axonal Caliber Associated With Mitochondrial Alterations in the White Matter of Chronically‐Starved Mice

International Journal of Eating Disorders, EarlyView.
ABSTRACT Objective Anorexia nervosa (AN) is a severe eating disorder associated with extreme weight loss, hyperactivity, and amenorrhea. Neuroimaging studies revealed brain atrophy and disruption of white matter integrity in the corpus callosum (CC) of patients with AN. However, the underlying pathophysiological mechanisms remain unclear.
Stephan Lang, Annelie Zimmermann, Kaja Dickert, Hanna Rupprecht, Julia Priebe, Fabienne Haberland, Hanna‐Sophia Henschke, Katharina Schuster, Marcus Frank, Linda Frintrop   +9 more
wiley   +1 more source

Insertion of mutant proteolipid protein results in missorting of myelin proteins [PDF]

Annals of Neurology, 2003
AbstractTwo brothers with a leukodystrophy, progressive spastic diplegia, and peripheral neuropathy were found to have proteinaceous aggregates in the peripheral nerve myelin sheath. The patients' mother had only subclinical peripheral neuropathy, but the maternal grandmother had adult‐onset leukodystrophy.
VAURS BARRIERE C, WONG K, WEIBEL TD, ABU ASAB M, WEISS MD, KANESKI CR, MIXON TH, BONAVITA, Simona, CREVEAUX I, HEISS JD, TSOKOS M, GOLDIN E, QUARLES RH, BOESPFLUG TANGUY O, SCHIFFMANN R.   +14 more
openaire   +3 more sources

Distinctive Structural and Molecular Features of Myelinated Inhibitory Axons in Human Neocortex. [PDF]

, 2018
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, Cobbs, Charles, Lein, Ed, Madison, Daniel V, Micheva, Kristina D, Nana, Alissa L, Seeley, William W, Smith, Stephen J, Ting, Jonathan T, Weinberg, Richard J   +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]

, 1989
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, Ben-Nun, Bostock, Brosnan, Brostoff, Carnegie, D.O. Willenborg, Deibler, Finean, Folch, Greefield, Heininger, Hoffman, K.B. Nguyen, Kerlero, Lassmann, Lassmann, Linington, M.P. Pender, Olsson, Panitch, Panitch, Paterson, Paterson, Pender, Pender, Pender, Pender, Pender, Pender, Pender, Pender, Pender, Pender, Pender, Pender, Raine, Raine, Richert, Satoh, Sedgwick, Simmons, Simmons, Smith, Smith, Tabira, Vandenbark, Waibl, White, Zamvil   +49 more
core   +1 more source

Olig2‐specific loss‐of‐function Slc35a2 results in hypomyelination and spontaneous seizures

Epilepsia, EarlyView.
Abstract Objective Malformations of cortical development represent major causes of drug‐resistant epilepsy, with mild malformation of cortical development with oligodendroglial hyperplasia and epilepsy recognized as a distinct pathological entity. Pathogenic X‐linked SLC35A2, encoding the uridine diphosphate–galactose transporter, has been implicated ...
Tiffany M. Bartel, Chaitali Ghosh, Kathryn Bisaha, Jean Khoury, Ashley Nemes‐Baran, Ingmar Blumcke, Imad Najm   +6 more
wiley   +1 more source

CDC42‐Effector Proteins Regulate Higher Order Structure of Septins Required for CNS Myelin Integrity

Glia, Volume 74, Issue 3, March 2026.
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, Joana Paes de Faria, Olaf Jahn, Ege Bilgin, Nikola Łukasik, Chethana Rao, Mišo Mitkovski, Fritz Benseler, Nils Brose, Sophie B. Siems, Sandra Goebbels, Wiebke Möbius, Helge Ewers, João B. Relvas, Hauke B. Werner   +14 more
wiley   +1 more source