Lipoprotein -> Fatty acid -> beta-oxidation in the mitochondria, which is carnitine carnitine-dependent step.
- If not, <u>Adipate and Suberate</u> are produced outside the mitochondria by peroxisomes.
- <u>Ethylmalonate</u> is a byproduct of the breakdown of butyrate. which can be accumulated by carnitine deficiency.
- Carnitine is biosynthesized from the amino acid lysine.
- The Vitamin B2(Riboflavin) is essential because it is the precursor to the coenzymes FMN and FAD which are crucial for the beta-oxidation of FA, the electron transport chain, and overall energy production.
- Fatty acid -> Fatty Acyl-CoA / Acetocetate <-> Acetyl CoA -> Citric cycle -> ATP
- So if these 3 organic acids are detected high in urine, L-lysine (if Low, 500mg tid), L-carnitine (500-1000mg TID), Vitamin B2(Riboflavin)(100mg TID) is recommended
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