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TDP-43 Pathology in ALS The pathological hallmarks of TDP-43 proteinopathies include nucleus to cytoplasmic mislocalization, deposition of ubiquitinated and hyper-phosphorylated TDP-43 into inclusion bodies, protein truncation leading to formation of toxic C-terminal TDP-43 fragments, and protein aggregation

However, affinity to NADH is substantially weakened compared to Peredox ( K d(NADH) 1.2) [23] and for each NAPstar is one to two orders of magnitude lower than for NADPH ( Figure 1D,E )