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

Author:

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

Funder

A Cure for Ellie

National Institutes of Health

NINDS

NICHD

Office of the NIH Director

Publisher

Elsevier BV

Subject

Developmental Neuroscience,Neurology

Reference46 articles.

1. A Wars2 mutant mouse model displays OXPHOS deficiencies and activation of tissue-specific stress response pathways;Agnew;Cell Rep.,2018

2. DARS2 protects against neuroinflammation and apoptotic neuronal loss, but is dispensable for myelin producing cells;Aradjanski;Hum.Mol.Genet.,2017

3. Mitochondria at the interface between neurodegeneration and neuroinflammation;Bader;Semin. Cell Dev. Biol.,2019

4. Leukoencephalopathy with brain stem and spinal cord involvement and lactate elevation is associated with cell-type-dependent splicing of mtAspRS mRNA;van Berge;Biochem. J.,2012

5. Pathogenic mutations causing LBSL affect mitochondrial aspartyl-tRNA synthetase in diverse ways;van Berge;Biochem. J.,2013

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