Article ; Online: S-Adenosyl-l-methionine restores brain mitochondrial membrane fluidity and GSH content improving Niemann-Pick type C disease.
2024 Volume 72, Page(s) 103150
Abstract: ... shown to contribute to the progression of NPC disease. S-Adenosyl-l-methionine (SAM) regulates membrane ...
Abstract | Niemann-Pick type C (NPC) disease is a lysosomal storage disorder characterized by impaired motor coordination due to neurological defects and cerebellar dysfunction caused by the accumulation of cholesterol in endolysosomes. Besides the increase in lysosomal cholesterol, mitochondria are also enriched in cholesterol, which leads to decreased membrane fluidity, impaired mitochondrial function and loss of GSH, and has been shown to contribute to the progression of NPC disease. S-Adenosyl-l-methionine (SAM) regulates membrane physical properties through the generation of phosphatidylcholine (PC) from phosphatidylethanolamine (PE) methylation and functions as a GSH precursor by providing cysteine in the transsulfuration pathway. However, the role of SAM in NPC disease has not been investigated. Here we report that Npc1 |
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Language | English |
Publishing date | 2024-04-03 |
Publishing country | Netherlands |
Document type | Journal Article |
ZDB-ID | 2701011-9 |
ISSN | 2213-2317 ; 2213-2317 |
ISSN (online) | 2213-2317 |
ISSN | 2213-2317 |
DOI | 10.1016/j.redox.2024.103150 |
Database | MEDical Literature Analysis and Retrieval System OnLINE |
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