To view a copy of this license, visit About this article Cite this article Mahon, K., Qu, W., Devaney, J
Amyotrophic lateral sclerosis (ALS) is a fatal, incurable and debilitating neurodegenerative condition that results in progressive degeneration and death of nerve cells and the eventual loss of the ability to eat, speak, move and breathe
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It inhibits NOTCH1 signaling by using its GCT activity to cleave a -glutamyl-cysteine bond at glutamate 1669 of the immature NOTCH receptor, thereby preventing the essential S1 cleavage and subsequent activation ( FIGURE 6 The physiological importance of this mechanism is underscored by severe developmental phenotypes upon its disruption
[PubMed: 17112576] 227
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