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By definition, in hyperammonemic encephalopathies circulating ammonia concentration is significantly increased, reaching high micromolar or even millimolar concentrations in UCDs (Michalak et al., 1996) and ALF patients (Clemmesen et al., 1999)

Although a single mechanism responsible for ARHL is yet to be identified, and is rather unlikely with the multifactorial and cumulative nature of the damage, it is certain that ongoing research efforts to advance our understanding of SNHL, for example with transgenic animal models, high-throughput genome sequencing and proteomics, will further support evidence-based treatment

Sci Signal 10:eaao6604 Lim JKM, Delaidelli A, Minaker SW, Zhang HF, Colovic M, Yang H, Negri GL, von Karstedt S, Lockwood WW, Schaffer P et al (2019) Cystine/glutamate antiporter xCT (SLC7A11) facilitates oncogenic RAS transformation by preserving intracellular redox balance

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Immunity 48 , 812830.e14 (2018)

S., Gothai, S., Fakurazi, S., Norhaizan, M