l glutathione jp monograph L-Glutathione reduced 500 mg MONO Glutathione | C10H17N3O6S | CID

10.1016/j.ogla.2024.07.008 [DOI] [PubMed] [Google Scholar] 208.Talwar N, Musch DC, Stein JD

Abbreviations: BSO, buthionine sulfoximine

TPP functions as a cofactor for several critical enzymes across three metabolic pathways: transketolase (TK) in the pentose phosphate pathway, pyruvate dehydrogenase (PDH) in glycolysis, and alpha-ketoglutarate dehydrogenase (AKD) in the Krebs cycle (, , )

[68] [69] Nicotinamide (vitamin B3) was found effective in preclinical FRDA models and well tolerated

It does not affect blood sugar or cause growth-related side effects

MDHAR4 mutants also have lower ascorbate to DHA ratios, but have similar total ascorbate levels to the wild type (Eastmond, 2007)