scholarly journals Making the White Matter Matters: Progress in Understanding Canavan’s Disease and Therapeutic Interventions Through Eight Decades

2014 ◽  
pp. 11-22 ◽  
Author(s):  
Seemin S. Ahmed ◽  
Guangping Gao
Keyword(s):  
White Matter ◽  
Therapeutic Interventions ◽  
Canavan's Disease ◽  
Canavan’S Disease

scholarly journals Aberrant Oligodendrogenesis in Down Syndrome: Shift in Gliogenesis?

Cells ◽  
2019 ◽  
Vol 8 (12) ◽  
pp. 1591 ◽  
Author(s):  
Laura Reiche ◽  
Patrick Küry ◽  
Peter Göttle
Keyword(s):  
Down Syndrome ◽  
White Matter ◽  
Cell Fate ◽  
Neuronal Development ◽  
Demyelinating Diseases ◽  
Therapeutic Interventions ◽  
Cell Lineages ◽  
Cns Malformations ◽  
Current Experience

Down syndrome (DS), or trisomy 21, is the most prevalent chromosomal anomaly accounting for cognitive impairment and intellectual disability (ID). Neuropathological changes of DS brains are characterized by a reduction in the number of neurons and oligodendrocytes, accompanied by hypomyelination and astrogliosis. Recent studies mainly focused on neuronal development in DS, but underestimated the role of glial cells as pathogenic players. Aberrant or impaired differentiation within the oligodendroglial lineage and altered white matter functionality are thought to contribute to central nervous system (CNS) malformations. Given that white matter, comprised of oligodendrocytes and their myelin sheaths, is vital for higher brain function, gathering knowledge about pathways and modulators challenging oligodendrogenesis and cell lineages within DS is essential. This review article discusses to what degree DS-related effects on oligodendroglial cells have been described and presents collected evidence regarding induced cell-fate switches, thereby resulting in an enhanced generation of astrocytes. Moreover, alterations in white matter formation observed in mouse and human post-mortem brains are described. Finally, the rationale for a better understanding of pathways and modulators responsible for the glial cell imbalance as a possible source for future therapeutic interventions is given based on current experience on pro-oligodendroglial treatment approaches developed for demyelinating diseases, such as multiple sclerosis.

Molecular Basis of Canavan's Disease

2003 ◽  
Vol 18 (9) ◽  
pp. 604-610 ◽  
Author(s):  
Sankar Surendran ◽  
Kimberlee M. Matalon ◽  
Stephen K. Tyring ◽  
Reuben Matalon
Keyword(s):  
Molecular Basis ◽  
Canavan's Disease ◽  
Canavan’S Disease

In vivo assessment of N-acetylaspartate in brain in spongy degeneration (Canavan's disease) by proton spectroscopy

The Lancet ◽  
1990 ◽  
Vol 336 (8712) ◽  
pp. 437-438 ◽  
Author(s):  
Wolfgang Grodd ◽  
Ingeborg Krägeloh-Mann ◽  
Dirk Petersen ◽  
Friedrich Karl Trefz ◽  
Klaus Harzer
Keyword(s):  
Proton Spectroscopy ◽  
Spongy Degeneration ◽  
Canavan's Disease ◽  
In Vivo Assessment ◽  
Canavan’S Disease

scholarly journals Altered oligodendroglia and astroglia in chronic traumatic encephalopathy

2020 ◽  
Author(s):  
K. Blake Chancellor ◽  
Sarah E. Chancellor ◽  
Joseph E. Duke-Cohan ◽  
Bertrand R. Huber ◽  
Thor D. Stein ◽  
...  
Keyword(s):  
White Matter ◽  
Chronic Traumatic Encephalopathy ◽  
Cell Types ◽  
Cellular Stress Response ◽  
Therapeutic Interventions ◽  
Cell Nuclei ◽  
Head Impacts ◽  
Starting Point ◽  
Cell Groups ◽  
And Control

Abstract Chronic traumatic encephalopathy (CTE) is a progressive tauopathy found in contact sport athletes, military veterans, and others exposed to repetitive head impacts (RHI)1–6. White matter atrophy and axonal loss have been reported in CTE but have not been characterized on a molecular or cellular level2,7,8. Here, we present RNA sequencing profiles of cell nuclei from postmortem dorsolateral frontal white matter from eight individuals with neuropathologically confirmed CTE and eight age- and sex-matched controls. Analyzing these profiles using unbiased clustering approaches, we identified eighteen transcriptomically distinct cell groups (clusters), reflecting cell types and/or cell states, of which a subset showed differences between CTE and control tissue. Independent in situ methods applied on tissue sections adjacent to that used in the single-nucleus RNA-seq work yielded similar findings. Oligodendrocytes were found to be most severely affected in the CTE white matter samples; they were diminished in number and altered in relative proportions across subtype clusters. Further, the CTE-enriched oligodendrocyte population showed greater abundance of transcripts relevant to iron metabolism and cellular stress response. CTE tissue also demonstrated excessive iron accumulation histologically. Astrocyte alterations were more nuanced; total astrocyte number was indistinguishable between CTE and control samples, but transcripts associated with neuroinflammation were elevated in the CTE astrocyte groups as compared to controls. These results demonstrate specific molecular and cellular differences in CTE oligodendrocytes and astrocytes and may provide a starting point for the development of diagnostics and therapeutic interventions.

scholarly journals Gene Therapy for Canavan's Disease Takes a Step Forward

2013 ◽  
Vol 21 (3) ◽  
pp. 505-506 ◽  
Author(s):  
Seemin S Ahmed ◽  
Guangping Gao
Keyword(s):  
Gene Therapy ◽  
Canavan's Disease ◽  
Canavan’S Disease
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