• Apolipoproteins are the protein components of the lipoproteins.

• Apolipoprotein B exists in two forms; apolipoprotein B-100

(apo B-100), which is made in the liver and is the structural
protein of VLDL, IDL and LDL, and apo B-48, which is synthesized in the
intestine and is incorporated into chy-lomicrons.
• Both forms of apo B are produced from the APOB gene;
Apolipoprotein B-100
Apolipoprotein B-100 (apo B-100) is necessary for secretion of VLDL.
It contains several very hydrophobic areas that serve as strong lipid-
binding domains.
apo B-100 contains an LDL receptor binding do-main (amino acids
3100–3400), which allows the specific uptake of LDL by the LDL
receptor.
Familial defective apolipoprotein B-100
(FDB).
• Familial defective apolipoprotein B-100 (FDB) is an autosomal co-
dominant genetic disorder of lipid metabolism caused by the
substitution of glutamine for arginine at position 3500 in apo B-100.
• FDB is caused by mutations in APOB reducing the binding affinity
between apolipoprotein B-100 and the low-density lipoprotein
receptor, leading to hypercholesterolaemia and increased proneness
to coronary artery disease..
• Low density lipoproteins accumulate in the plasma because their
efficient receptor-mediated catabolism is disrupted.