Pathogenesis of leukodystrophy for Krabbe disease: Molecular mechanism and clinical treatment

2009 ◽  
Vol 31 (7) ◽  
pp. 485-487 ◽  
Author(s):  
Norio Sakai
2020 ◽  
Author(s):  
Liuliu Yang ◽  
Minyong Wen ◽  
Wenjiang Zheng ◽  
Xiaohong Liu ◽  
yong wang

Abstract Background: This paper discusses the molecular mechanism of Tanreqing (TRQ) in the treatment of the coronavirus disease 2019 (COVID-19) using the network pharmacology approach. Our study provides new ideas on the laboratory research and clinical treatment of the disease. Method: Information on the chemical constituents of TRQ and the genes targeted by the disease was collected. The common gene targets of the drug and the disease were input into the Search Tool for the Retrieval of Interacting Genes/Proteins(STRING)database to understand the interaction among target proteins. The protein–protein interaction (PPI) network and a network of the chemical constituents of TRQ and their targets were constructed using Cytoscape. Gene ontology (GO) enrichment analysis and Kyoto Encyclopedia of Genes and Genomes (KEGG) pathway analysis were performed using the R program and other relevant software packages. Results: Twenty-eight active chemical constituents and 365 gene targets were identified for TRQ. Out of these genes, 113 were also found to be involved in the pathogenesis of the disease. Enrichment analysis revealed the therapeutic role that TRQ could have played in the treatment of COVID-19, via the regulation of important pathways such as the renin–angiotension system, neuroactive ligand–receptor interaction, phospholipase D (PLD) signaling pathway, calcium signaling pathway, and the hypoxia-inducible factor 1 (HIF-1) signaling pathway. Conclusions: This study attempts to predict the molecular mechanism of TRQ in the treatment of COVID-19, and suggests TRQ intervention through multiple targets and pathways in processes including inflammatory response, immune regulation, and apoptosis during the treatment of the disease. This study indicates the potential rational application of TRQ in the clinical treatment of COVID-19.


2021 ◽  
Vol 8 ◽  
Author(s):  
Gibran Nasir ◽  
Rajiv Chopra ◽  
Fiona Elwood ◽  
Seemin S. Ahmed

Krabbe Disease (KD) is an autosomal metabolic disorder that affects both the central and peripheral nervous systems. It is caused by a functional deficiency of the lysosomal enzyme, galactocerebrosidase (GALC), resulting in an accumulation of the toxic metabolite, psychosine. Psychosine accumulation affects many different cellular pathways, leading to severe demyelination. Although there is currently no effective therapy for Krabbe disease, recent gene therapy-based approaches in animal models have indicated a promising outlook for clinical treatment. This review highlights recent findings in the pathogenesis of Krabbe disease, and evaluates AAV-based gene therapy as a promising strategy for treating this devastating pediatric disease.


2021 ◽  
Author(s):  
Sun Jing ◽  
Xianyang Chen ◽  
Bei Fan ◽  
Bo Pan ◽  
Jiani Liu ◽  
...  

Abstract Background:Longxuetongluo Capsule (LTC) that was prepared from the phenolic compound cluster of Chinese dragon’s blood, has been clinically utilized for years towards ischemic stroke. However, its therapeutic mechanism s remain unclear. The present study aimed to evaluate the therapeutic effect and to explore the underlying mechanisms of LTC on ischemic stroke rats characterized by cerebral ischemia 6 re perfusion injury. Method:Ischemic stroke rats were simulated by middle cerebral artery occlusion ( and validated by the assays in terms of histological and behavioral assessments . The plasma metabolome comparisons amongst different groups were conducted by UHPLC Q Executive MS in combination with subsequent multivariate statistical analysis , aiming to finding the molecules in respond to the surgery or LTC treatment . Result:Intragastric administration of LTC significantly decreased not only the neurobehavioral abnormality scores but also the cerebral infarct area of MCAO rats. A total of 21 potential biomarkers related to ischemic stroke were observed, and 18 biomarkers (such as LysoPC, PE, SM, N1-Methyl-4-pyridone-3-carboxamide, PE (24:1/18:3 etc.) were selected after Youden index >0.7. The results showed that LTC was beneficial to ischemic stroke rats induced by ischemia reperfusion injury by Glycerophospholipid metabolism , Glycosylphosphatidylinositol (GPI) anchor biosynthesis , Nicotinate and nicotinamide metabolism and Sphingolipid metabolism Conclusion:The present study could provide evidence that metabolomics, as system systematicatic approach, revealed its capacity to evaluate tapproach, revealed its capacity to evaluate the holistic efficacy of TCM, he holistic efficacy of TCM, and investigate the molecular mechanism underlying the clinical treatment of LTC on and investigate the molecular mechanism underlying the clinical treatment of LTC on ischemic stroke. ischemic stroke.


2001 ◽  
Vol 120 (5) ◽  
pp. A91-A91
Author(s):  
P TIETZ ◽  
P SPLINTER ◽  
M MCNIVEN ◽  
R HUEBERT ◽  
N LARUSSO

1998 ◽  
Vol 5 (1) ◽  
pp. 187A-187A
Author(s):  
J CARVAJAL ◽  
S KATO ◽  
J SAEZ ◽  
F LEIGHTON ◽  
G VALENZUELA ◽  
...  

2008 ◽  
Vol 78 (1) ◽  
pp. 3-8 ◽  
Author(s):  
Fan ◽  
Jiang ◽  
Zhang ◽  
Bai

In efforts to identify naturally occurring compounds that act as protective agents, resveratrol, a phytoalexin existing in wine, has attracted much interest because of its diverse pharmacological characteristics. Considering that apoptosis induction is the most potent defense approach for cancer treatment, we have tried to summarize our present understanding of apoptosis induction by resveratrol based on the two major apoptosis pathways.


1987 ◽  
Vol 32 (12) ◽  
pp. 1036-1036
Author(s):  
Susan Krauss Whitbourne

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