Neuronal ablation of mt-AspRS in mice induces immune pathway activation prior to severe and progressive cortical and behavioral disruption

Leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation (LBSL) is a rare, slowly progressive white matter disease caused by mutations in the mitochondrial aspartyl-tRNA synthetase (mt-AspRS, or DARS2). While patients show characteristic MRI T2 signal abnormalities throu...

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Pubblicato in:Exp Neurol
Autori principali: Nemeth, Christina L, Tomlinson, Sophia N, Rosen, Melissa, O’Brien, Brett M, Larraza, Oscar, Jain, Mahim, Murray, Connor F, Marx, Joel S, Delannoy, Michael, Fine, Amena S, Wu, Dan, Trifunovic, Aleksandra, Fatemi, Ali
Natura: Artigo
Lingua:Inglês
Pubblicazione: 2019
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Article
Accesso online:https://ncbi.nlm.nih.gov/pmc/articles/PMC7448750/
https://ncbi.nlm.nih.gov/pubmed/31887305
https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1016/j.expneurol.2019.113164
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