Loss of Nardilysin, a Mitochondrial Co-chaperone for α-Ketoglutarate Dehydrogenase, Promotes mTORC1 Activation and Neurodegeneration
文摘
NRD1 binds to and chaperones OGDH (α-KGDH) in mitochondria Loss of NRD1 or OGDH leads to a progressive neurodegeneration Loss of NRD1 or OGDH increases α-KG, elevates mTORC1 activity, and impairs autophagy Rapamycin alleviates neuronal defects resulting from reduced NRD1 or OGDH function