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doi: 10.1002/hep.28487 85

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

How lipo battery's performance affected by temperature

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)