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. Author manuscript; available in PMC: 2020 Oct 1.
Published in final edited form as: Mol Cell Neurosci. 2019 Aug 21;100:103396. doi: 10.1016/j.mcn.2019.103396

Fig.1. TDP-43 proteinopathy and mitochondrial abnormalities in neurodegeneration.

Fig.1.

(A) TDP-43 mitochondrial association. OMM, outer mitochondrial membrane; IMM, inner mitochondrial membrane; IMS, intermembrane space. (B) Mitochondria are dynamic organelles that undergo continuous fission, fusion, and trafficking, which are critical for the maintenance of mitochondrial function in neurons. The majority of TDP-43 resides in the nuclei and involves in a wide range of cellular processes such as RNA processing, cryptic splicing, RNA transport, and microRNA biogenesis in healthy neurons. (C) In disease-affected neurons, TDP-43 forms inclusions in the cytoplasm, usually accompanied by depletion of nuclear TDP-43. Abnormal mitochondria with altered morphology, inner structure, and OXPHOS activity can be noted in neurons bearing TDP-43 proteinopathy.