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The first three enzymes of carnitine endogenous biosynthesis, -N-trimethyllysine hydroxylase, -hydroxy-N--trimethyllysine aldolase, and 4-N-trimethylaminobutyraldehyde dehydrogenase are widely distributed, while butyrobetaine hydroxylase is only present in kidney (highest enzyme activity), liver and brain (lowest measurable enzyme activity)

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