PHSI3012 10-1 Disorders of Lipid Metabolism #1 (T3L1)

Free fatty acids (NEFA / Non-Esterified Fatty Acids) vs TriAcylGlycerides (/TAGs).
❌ circulate bound to albumin.
❌ circulate within ❌ released from the liver or ❌ released from the gut.

Lipid uptake.
Put the three in order (they’re written alphabetically here)
❌ Cholesterol-rich LDL interact with LDL-R and scavenger receptors; enters lysosomal pathway for hydrolysis;
❌ Hepatic Lipase and Lipoprotein Lipase reside on endothelial cells to hydrolyse VLDL and Chylomicron TAG to FFAs for uptake;
❌ TAG-rich VLDL interact with VLDL-R, enters lysosomal pathway where acid lipases hydrolyse TAG.

LPL deficiency causes ❌, which leads to ❌.

De Novo Fatty Acid Synthesis uses ❌ (from Glucose and Amino acids) to generate ❌ as the starting substrate to firstly produce ❌ (C16:O).

MCD deficiency (and some similar disorders) prevent ❌ from being turned into ❌. This results in ❌ of CPT1 (and thus ❌ FA uptake).