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FASEB J 27: 174186 Smethurst P, Newcombe J, Troakes C, Simone R, Chen YR, Patani R, Sidle K (2016) In vitro prionlike behaviour of TDP43 in ALS

Science 364 , 552557 (2019)

However, we found that both Alanine and Cystine substantially fell outside of healthy reference ranges, with Cystine being dramatically reduced in both affected and unaffected siblings with average plasma concentrations of 3.0 mol/L and 3.3 mol/L respectively, while Alanine was found to be elevated regardless of disease status (U:595.5 mol/L, K:589.0 mol/L)

Patients on dialysis received a higher concentration of sodium ascorbate (3 g) due to high clearance due to dialysis, under the same conditions as patients without renal replacement

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Treatment increased beneficial Bacteroides acidifaciens and reduced pathogenic Clostridiales bacterium and Proteobacteria