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a) Lipoproteins are large molecular compounds that are essential to the transport of cholesterol and triglycerides (TGs) within the blood. They contain a lipid core composed of TGs and cholesterol esters surrounded by phospholipids and specialized proteins known as apolipoproteins. The five major families of lipoprotein are chylomicrons, very-low-density lipoproteins (VLDL), intermediate-density lipoproteins (IDL), low-density lipoproteins (LDL), and high-density lipoproteins (HDL).
b) Apolipoproteins are necessary for the structure and enzymatic processes of lipids. Apolipoprotein A1 (apo A1) is a major component of HDL, and apolipoprotein B (apo B) is the main apolipoprotein for the remaining non-HDL lipoproteins.
The diet of a MSUD patient (child) should therefore involve: • Measured quantities of natural protein or leucine from foods. • A BCAA free protein, vitamin and mineral supp
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