The levels of TDP-43, another protein showing pathological accumulation in FTLD brain [ 71 , 72 ], were significantly increased in fibroblasts without the C9-HRE and also showed a trend toward increased levels in fibroblasts with the C9-HRE compared to controls but did not show alterations after induction of autophagy with Torin 1 (Fig. 4a, i ).
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FTLD Patient-Derived Fibroblasts Show Defective Mitochondrial Function and Accumulation of p62.
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