What is the role of beta apolipoproteins in the pathophysiology of abetalipoproteinemia (ABL)?

Updated: Mar 06, 2018
  • Author: Vibhuti N Singh, MD, MPH, FACC, FSCAI; Chief Editor: George T Griffing, MD  more...
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Beta apolipoproteins are the largest of the apolipoproteins. They are critically important for the formation and secretion of CMs and VLDL; abnormalities that impede this process result in abetalipoproteinemia (ABL) and hypobetalipoproteinemia.

The 2 beta apolipoproteins are B-100 and B-48. ApoB-100 is carried on VLDL and the lipoproteins derived from its metabolism, including VLDL remnants or intermediate-density lipoprotein and LDL. ApoB-100, which is synthesized by the liver, is larger than apoB-48, being made up of 4536 amino acids. Unlike apoB-48, apoB-100 contains the binding site essential for LDL uptake by hepatocyte LDL receptors. [9] ApoB-48 is carried on CMs, is derived from the same gene as apoB-100, and is approximately half its size, consisting of 2152 amino acids.

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