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For instance, in patients with maple syrup urine disease (MSUD), an inborn error of metabolism caused by loss-of-function mutation in components of the BCKD complex [123,124,125,126] or its regulatory phosphatase, PPM1K [127], BCAA levels in plasma are found to be elevated

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3.2 Liver Diseases Mitochondrial FAO is the primary pathway for FA metabolism and performs a key role for energy homeostasis in the liver (Li and Davie, 2010

(ii) In human studies, this includes differences in race and patient age, as well as sex, diet, and drug use, which can cause changes in the composition of the gut bacterial microbiota and alter the relationship between plasma TMAO levels and atherosclerosis