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Proc Natl Acad Sci USA 111(28):1020910214

The two Asp's act as the main catalytic agents, and together with a water molecule cleave the protein chain bound in the tunnel

These smaller fluctuations are a result of the inhibition of the GCS reaction by GSH (see Section C)

In terms of clinical subtypes, UMN-predominant ALS shows greater cortical hypointensity compared to LMN-dominant forms, indicating specificity in iron distribution (342, 343)