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In 2024, a study using untargeted metabolomics compared propionic acidemia, cobalamin C disease and methylmalonic acidemia, showing major perturbation of the Krebs cycle, glycine metabolism, and oxidative stress in methylmalonic acidemia (152)

However, future study should determine liver function test and urinary albumin excretion to confirm the reason of reduced serum total protein in MSG treated group

Attia AA, Hamad HA, Fawzy MA, Saleh SR (2023) The Prophylactic Effect of Vitamin C and Vitamin B12 against Ultraviolet-C-Induced Hepatotoxicity in Male Rats

In skeletal muscle of UBE3B KO mice, there is an accumulation of BDK that leads to a decrease in BCKDH complex activity, but this does not correlate with changes in BCAA levels, indicating that BCKAs could be driven to other pathways (Ziegler et al., 2017), therefore BDK accumulation may disrupt the regulation of lipogenesis in developing neurons

India 56 , 420425 (2008)