scholarly journals Simulating the enzymes of ganglioside biosynthesis with Glycologue

2021 ◽  
Vol 17 ◽  
pp. 739-748
Author(s):  
Andrew G McDonald ◽  
Gavin P Davey
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cell Surface ◽  
Network Structure ◽  
Metabolic Pathways ◽  
Congenital Defects ◽  
Important Class ◽  
Specific Enzyme ◽  
Ganglioside Biosynthesis ◽  
The Central Nervous System

Gangliosides are an important class of sialylated glycosphingolipids linked to ceramide that are a component of the mammalian cell surface, especially those of the central nervous system, where they function in intercellular recognition and communication. We describe an in silico method for determining the metabolic pathways leading to the most common gangliosides, based on the known enzymes of their biosynthesis. A network of 41 glycolipids is produced by the actions of the 10 enzymes included in the model. The different ganglioside nomenclature systems in common use are compared and a systematic variant of the widely used Svennerholm nomenclature is described. Knockouts of specific enzyme activities are used to simulate congenital defects in ganglioside biosynthesis, and altered ganglioside status in cancer, and the effects on network structure are predicted. The simulator is available at the Glycologue website, https://glycologue.org/.

scholarly journals Detection and Characterization of Autoantibodies to Neuronal Cell-Surface Antigens in the Central Nervous System

2016 ◽  
Vol 9 ◽  
Author(s):  
Marleen H. van Coevorden-Hameete ◽  
Maarten J. Titulaer ◽  
Marco W. J. Schreurs ◽  
Esther de Graaff ◽  
Peter A. E. Sillevis Smitt ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cell Surface ◽  
Neuronal Cell ◽  
Surface Antigens ◽  
Cell Surface Antigens ◽  
The Central Nervous System

scholarly journals Loss of PRMT5 promotes PDGFRα degradation during oligodendrocyte differentiation and myelination

2018 ◽  
Author(s):  
Sara Calabretta ◽  
Gillian Vogel ◽  
Zhenbao Yu ◽  
Karine Choquet ◽  
Lama Darbelli ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Growth Factor ◽  
Cell Surface ◽  
Growth Factor Receptor ◽  
Arginine Methylation ◽  
Platelet Derived Growth Factor ◽  
Oligodendrocyte Differentiation ◽  
The Central Nervous System ◽  
Cns Myelination

SummaryPlatelet derived growth factor receptor α (PDGFRα) signaling is required for proliferation, commitment and maintenance of oligodendrocyte (OL) precursor cells (OPCs). PDGFRα signaling promotes OPC homeostasis and its attenuation signals OPC differentiation and maturation triggering the onset of myelination of the central nervous system (CNS). The initial steps of how PDGFRα signaling is attenuated are still poorly understood. Herein we show that decreased Protein Arginine MethylTransferase5 (PRMT5) expression, as occurs during OPC differentiation, is involved in the down-regulation of PDGFRα by modulating its cell surface bioavailability leading to its degradation in a Cbldependent manner. Mechanistically, loss of arginine methylation at R554 of the PDGFRα intracellular domain reveals a masked Cbl binding site at Y555. Physiologically, depletion of PRMT5 in OPCs results in severe CNS myelination defects. We propose that decreased PRMT5 activity initiates PDGFRα degradation to promote OL differentiation. More broadly, inhibition of PRMT5 may be used therapeutically to manipulate PDGFRα bioavailability.

scholarly journals Serum metallome in pregnant women and the relationship with congenital malformations of the central nervous system: a case-control study

2019 ◽  
Vol 19 (1) ◽  
Author(s):  
Jacopo Troisi ◽  
Luigi Giugliano ◽  
Laura Sarno ◽  
Annamaria Landolfi ◽  
Sean Richards ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Inductively Coupled Plasma ◽  
Congenital Malformations ◽  
Limit Of Detection ◽  
Maternal Serum ◽  
Congenital Defects ◽  
Inductively Coupled ◽  
Wide Range ◽  
The Central Nervous System

Abstract Background Congenital malformations of the central nervous system (CNS) consist of a wide range of birth defects of multifactorial origin. Methods Concentrations of 44 metals were determined by Inductively Coupled Plasma Mass Spectrometry in serum of 111 mothers in the second trimester of pregnancy who carried a malformed fetus and compared them with serum concentrations of the same metals in 90 mothers with a normally developed fetus at the same week of pregnancy. Data are reported as means ± standard deviations. Results We found a direct relationship between congenital defects of the CNS and maternal serum concentration of aluminum: it was statistically higher in women carrying a fetus with this class of malformation, compared both to mothers carrying a fetus with another class of malformation (6.45 ± 15.15 μg/L Vs 1.44 ± 4.21 μg/L, p < 0.0006) and to Controls (i.e. mothers carrying a normally-developed fetus) (6.45 ± 15.15 μg/L Vs 0.11 ± 0.51 μg/L, p < 0.0006). Moreover, Aluminum abundances were below the limit of detection in the majority of control samples. Conclusion CAluminum may play a role in the onset of central nervous system malformations, although the exact Aluminum species and related specific type of malformation needs further elucidation.

Birth Defects in Twins: Study in a Spanish Population

1991 ◽  
Vol 40 (3-4) ◽  
pp. 337-344 ◽  
Author(s):  
M.A. Ramos-Arroyo
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Birth Defects ◽  
Congenital Malformations ◽  
Collaborative Study ◽  
Congenital Defects ◽  
Relative Risks ◽  
Increased Risk ◽  
The Central Nervous System ◽  
Using Data

AbstractThe risk for specific defects among twins compared to singletons was studied using data collected by the Spanish Collaborative Study of Congenital Malformations (ECEMC). A total of 136 twins had a major and/or minor congenital defect. The overall rate of congenital defects in twins (2.37%) did not deviate significantly from the rate in singletons (2.21%). Like-sex (LS) and male-male (MM) twin pairs had a slightly higher rate of birth defects than unlike-sex (US) and female-female (FF) pairs, respectively. Defects of the central nervous system, cardiovascular system and genitourinary system were significantly more frequent in LS twins than in singletons, with relative risks of 2.8, 2.5 and 1.6, respectively. No significantly increased risk was found among US twins. Among defects of the central nervous system, the rates of anencephaly, encephalocele and hydrocephaly were significantly higher in total and LS twins; however, no significantly increased risk for spina bifida was observed when compared to singletons. MM twins were also 1.9 times more likely to have hypospadias, but the risk among males of male-female (FM) pairs was decreased.

HVEM of Central Nervous System Tissue: Localization of the Site of Physiologic Measures

1985 ◽  
Vol 43 ◽  
pp. 586-587
Author(s):  
J. N. Turner ◽  
J. W. Swann ◽  
R. J. Brady ◽  
D. O. Carpenter ◽  
D. N. Collins
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Cell Surface ◽  
Surrounding Tissue ◽  
Central Nervous System Tissue ◽  
Lower Vertebrate ◽  
Preparation Methods ◽  
The Central Nervous System ◽  
Physiologic Parameter ◽  
Simple Systems

The correlation of physiology and ultrastructure is critical to many areas of neurobiology, but is usually possible only in “simple” systems, (neuromuscular junction, isolated invertebrate or lower vertebrate preparation or in culture). Such correlation is difficult in the central nervous system (CNS) due to its complex microanatomy, large number of synapses on each neuron and lack of techniques for finding specific sites, (e.g. presynaptic terminals buried in a mass of tissue). We are developing specimen labeling and preparation methods to mark the site at which a physiologic parameter has been measured or manipulated by a microelectrode. The marker must be either electron dense or fluorescent - the former is preferred. It must bind to the cell surface without excessive diffusion into surrounding tissue, and it must be visible during block trimming and sectioning, to form a traceable link from the macroscopic to the ultrastructure.

scholarly journals Composite bottlebrush mechanics: α-internexin fine-tunes neurofilament network properties

Soft Matter ◽  
2015 ◽  
Vol 11 (29) ◽  
pp. 5839-5849 ◽  
Author(s):  
M. Kornreich ◽  
E. Malka-Gibor ◽  
A. Laser-Azogui ◽  
O. Doron ◽  
H. Herrmann ◽  
...  
Keyword(s):  
Central Nervous System ◽  
Nervous System ◽  
Elastic Properties ◽  
Network Structure ◽  
Developmental Stages ◽  
Regulatory Mechanism ◽  
Early Developmental Stages ◽  
The Central Nervous System ◽  
Network Properties ◽  
Early Developmental

We measure the mechano-elastic properties of composite bottlebrush nematic hydrogels consisting of proteins originated from the central nervous system. We show that intra-brush interactions fine-tunes the network structure which may serve as a structural-regulatory mechanism in neuronal early developmental stages.

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