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

How many ATPs go where? (btw, "1 ATP" goes before "1ATP")

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

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

Familial Lipoprotein Lipase Deficiency is:

LPL deficiency causes [blank_start]__________[blank_end], which leads to [blank_start]__________[blank_end].

Fatty Acid Metabolism has two major pathways: TriAcylGlycerol metabolism and β-Oxidation. FA uptake is a 2-step process. In which order does it happen?

FA activation:

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

De Novo Fatty Acid Synthesis uses up how many ATP molecules?

CPT1 turns Carnitine into FA-Carnitine, allowing it to be uptaken into a cell. This can be blocked by which of the following?

MCD deficiency (and some similar disorders) prevent [blank_start]__________[blank_end] from being turned into [blank_start]__________[blank_end]. This results in [blank_start]__________[blank_end] of CPT1 (and thus [blank_start]__________[blank_end] FA uptake).

What goes where? (within each box, put the two in alphabetical order)