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Extraction of the intracellular metabolites Intracellular metabolites were extracted as reported previously [46, 47] with some modifications

High-dose B6 supplementation may be beneficial in the case of partial CBS activity to enhance residual enzyme function, while total enzyme deficiency requires a methionine-restricted diet, cysteine-supplemented diets and betaine, folate and B12 supplementation to promote alternate remethylation pathways (19,20)

They also have harmful effects on embryo development (Pietrzik and Bronstrup, 1997

doi: 10.1007/s40620-021-01007-z 70 LiuBCTangTTLvLLLanHY

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Next, to determine if ABHD11 acted directly on the Lp glu adduct in an enzymatic manner, we measured the activity of purified ABHD11 against a recombinant Lp glu OGDHc-E2 peptide encompassing the minimal residues around the lipoylated K110 (Extended Data Fig