Results 31 to 40 of about 5,666,125 (336)

Glial Fibrillary Acidic Protein Astrocytopathy Based on a Two‐Center Chinese Cohort Study

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective Glial fibrillary acidic protein astrocytopathy (GFAP‐A) is a recently defined nosological form belonging to the class of autoimmune inflammatory disorders affecting the central nervous system (CNS). Here, we report the clinical and MRI characteristics, treatment, and prognosis of a GFAP‐A cohort from two centers in China.
Ti Wu, Hao Zhang, Chao Gao, Qiuhua Yu, Moli Fan, Lin‐Jie Zhang, Haipeng Zhang, Hengri Cong, Yuzhen Wei, Chotima Böttcher, Alexej Verkhratsky, Friedemann Paul, Fu‐Dong Shi, Tian Song   +13 more
wiley   +1 more source

Repeat Expansions in PLIN4 Cause Autosomal Dominant Vacuolar Myopathy With Sarcolemmal Features

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objective We aim to describe and characterize two unrelated Spanish families suffering from an autosomal dominant autophagic vacuolar myopathy caused by repeat expansions in PLIN4. Methods We evaluated the clinical phenotype and muscle imaging, and performed a genetic workup that included exome sequencing, muscle RNAseq, and long‐read genome ...
Laura Llansó, Igor Stevanovski, Germán Morís, Roger Collet‐Vidiella, Alba Segarra‐Casas, Lidia González‐Quereda, Benjamín Rodríguez‐Santiago, Pia Gallano, Rodrigo Alvarez, Ana Vesperinas, Rosa Blanco, Beatriz San‐Millán, Carmen Navarro, Isabel Illa, Gianina Ravenscroft, Ira W. Deveson, Eduard Gallardo, Montse Olivé   +17 more
wiley   +1 more source

Turnover of myelin proteins in mouse brain in vivo.

Biochemical Journal, 1977
The incorporation of tyrosine into proteins was measured after the subcutaneous implantation of a pellet of [14C]tyrosine in mice. This method keeps the specific radioactivity of free tyrosine fairly constant and makes it possible to follow incorporation
A. Lajtha, J. Toth, K. Fujimoto, H. Agrawal   +3 more
semanticscholar   +1 more source

Nanoscale correlated disorder in out-of-equilibrium myelin ultrastructure

, 2017
Ultrastructural fluctuations at nanoscale are fundamental to assess properties and functionalities of advanced out-of-equilibrium materials. We have taken myelin as a model of supramolecular assembly in out-of-equilibrium living matter.
Bianconi, Antonio, Burghammer, Manfred, Campi, Gaetano, Ciasca, Gabriele, Di Gioacchino, Michael, Poccia, Nicola, Ricci, Alessandro   +6 more
core   +1 more source

A co-ultramicronized palmitoylethanolamide/luteolin composite mitigates clinical score and disease-relevant molecular markers in a mouse model of experimental autoimmune encephalomyelitis [PDF]

, 2019
Background: Persistent and/or recurrent inflammatory processes are the main factor leading to multiple sclerosis (MS) lesions. The composite ultramicronized palmitoylethanolamide, an endogenous N-acylethanolamine, combined with the flavonoid luteolin ...
Barbierato, M., Contarini, G., Facci, L., Franceschini, D., Giusti, P., Zusso, M.   +5 more
core   +1 more source

Polycistronic Delivery of IL-10 and NT-3 Promotes Oligodendrocyte Myelination and Functional Recovery in a Mouse Spinal Cord Injury Model. [PDF]

, 2020
One million estimated cases of spinal cord injury (SCI) have been reported in the United States and repairing an injury has constituted a difficult clinical challenge.
Anderson, Aileen J, Ciciriello, Andrew J, Cummings, Brian J, Dumont, Courtney M, Guo, Amina, Park, Jonghyuck, Shea, Lonnie D, Smith, Dominique R, Tatineni, Ravindra   +8 more
core   +1 more source

Central Dysmyelination in SSADH‐Deficient Humans and Mice

Annals of Clinical and Translational Neurology, EarlyView.
ABSTRACT Objectives Succinic semialdehyde dehydrogenase deficiency (SSADHD) is an inherited metabolic disorder characterized by an accumulation of γ‐aminobutyric (GABA). In addition to its synaptic role as an inhibitory neurotransmitter, GABA also plays an important role in myelination.
Itay Tokatly Latzer, Henry H. C. Lee, Edward Yang, Cesar Alves, Mariarita Bertoldi, Caitlyn Fung, Spencer V. Steele, Eren Kule, Zijie Jin, Alexander Rotenberg, Jean‐Baptiste Roullet, Phillip L. Pearl   +11 more
wiley   +1 more source

MENGA: a new comprehensive tool for the integration of neuroimaging data and the Allen human brain transcriptome atlas [PDF]

, 2016
Brain-wide mRNA mappings offer a great potential for neuroscience research as they can provide information about system proteomics. In a previous work we have correlated mRNA maps with the binding patterns of radioligands targeting specific molecular ...
Bertoldo, A, Expert, P, Rizzo, G, Turkheimer, FE, Veronese, M   +4 more
core   +4 more sources

Investigating the Cellular Effects of GALC Dosing in Enzyme Replacement Therapy for Krabbe Disease Supports the Role of Nanomedicine

Advanced Biology, EarlyView.
A detailed workflow for recombinant GALC production and characterization is presented to support enzyme replacement therapy for Krabbe disease. In vitro assays demonstrate that physiological GALC doses restore enzymatic activity and autophagic flux without affecting cell viability, whereas higher doses impair autophagy and reduce viability.
Ambra Del Grosso, Sara Carpi, Laura Colagiorgio, Miriam De Sarlo, Mariacristina Gagliardi, Marco Cecchini   +5 more
wiley   +1 more source

The Genetic Signature of Perineuronal Oligodendrocytes [PDF]

, 2008
Oligodendrocytes in the central nervous system can be categorized as precursors, myelin-forming, and non-myelinating perineuronal cells. The function of perineuronal oligodendrocytes is unknown; it was proposed that following injury, they may remyelinate
Dragan Maric, Gabor Lovas, Javier Martinez de Velasco, Joseph A. Nielsen, Lynn D. Hudson, Sara Szuchet   +5 more
core   +1 more source