Lipids 2
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73 Terms
What is steatorrhea?
Excess fat in stool
What are the two major lipid disorders discussed in this lecture?
Steatorrhea and hyperlipidemias
What is the function of bile in lipid digestion?
Emulsification
Where is bile produced?
Liver
Where is bile stored and released from?
Gallbladder
What are the products of pancreatic lipase action on TAGs?
2-MAG and 2 fatty acids
Which enzymes digest short- and medium-chain fatty acids?
Lingual and gastric lipases
Which fatty acids are hydrolyzed by lingual and gastric lipases?
What is the product of cholesterol ester digestion?
Cholesterol and fatty acid
Which enzyme digests cholesterol esters?
Pancreatic esterase
Which enzyme removes the fatty acid at C2 of phospholipids?
Phospholipase A2
What is the product of phospholipase A2 action?
Lysophospholipid
How are short-chain fatty acids transported in blood?
Bound to albumin
How are medium-chain fatty acids transported in blood?
Bound to albumin
Which fatty acids enter the portal blood directly?
Short- and medium-chain fatty acids
Which fatty acids require chylomicrons for transport?
Long-chain fatty acids
What is required for TAG re-synthesis in enterocytes?
2-MAG and LCFA-CoA
Which enzyme esterifies cholesterol in enterocytes?
ACAT
What does ACAT stand for?
Acyl-CoA cholesterol acyltransferase
Which apolipoprotein is synthesized in enterocytes?
Apo B-48
Which lipoprotein is formed in intestinal cells?
Chylomicron
What are the major components of chylomicrons?
TAGs, CE, PL, Apo B-48
Which apolipoproteins are added to chylomicrons by HDL?
Apo C-II and Apo E
What causes steatorrhea due to bile deficiency?
Impaired emulsification
What causes steatorrhea due to pancreatic disease?
Reduced lipid digestion
What causes steatorrhea due to intestinal disease?
Reduced lipid absorption
Which lipids are affected by bile deficiency?
TAGs, CE and phospholipids
Which lipids are affected by pancreatic insufficiency?
TAGs, CE and phospholipids
Which lipids are affected by absorptive defects?
Fatty acids, cholesterol and lysophospholipids
What is hyperlipidemia?
Elevated blood lipids
What is another name for Type I hyperlipoproteinemia?
Chylomicron syndrome
What enzyme deficiency commonly causes chylomicron syndrome?
Lipoprotein lipase deficiency
How common is lipoprotein lipase deficiency?
1 in 1,000,000
What retinal finding is associated with chylomicron syndrome?
Lipaemia retinalis
What is the main treatment for lipoprotein lipase deficiency?
Low-fat diet
What fat intake is recommended in LPL deficiency?
Which triglycerides are supplemented in LPL deficiency?
Medium-chain triglycerides
Which essential fatty acid should be supplemented in LPL deficiency?
Linoleic acid
What deficiency can be temporarily treated with fresh plasma?
Apo C-II deficiency
What is the most common inherited hyperlipidemia?
Familial hypercholesterolemia
How common is familial hypercholesterolemia?
1 in 500
Which populations have high rates of familial hypercholesterolemia?
Africans and French Canadians
What is the primary defect in familial hypercholesterolemia?
LDL receptor mutation
What is the most common lipid abnormality in familial hypercholesterolemia?
Increased LDL cholesterol
Name mechanisms causing LDL receptor dysfunction.
Reduced synthesis, Failed Golgi transport, Defective LDL binding, Inadequate expression, Defective receptor recycling
What tendon lesion is characteristic of familial hypercholesterolemia?
Tendinous xanthomas
What eye finding is associated with familial hypercholesterolemia?
Corneal arcus
What causes familial defective ApoB-3500?
ApoB gene mutation
Which amino acid substitution occurs in familial defective ApoB-3500?
Arginine replaced by glutamine
Which disease can familial defective ApoB-3500 resemble clinically?
Familial hypercholesterolemia
How is familial defective ApoB-3500 treated?
Same as familial hypercholesterolemia
What is another name for familial dysbetalipoproteinemia?
Broad beta disease
What Fredrickson type is familial dysbetalipoproteinemia?
Type III
Which apolipoprotein is abnormal in familial dysbetalipoproteinemia?
Apo E
Which Apo E variant is associated with dysbetalipoproteinemia?
Apo E2
What is the defect in Apo E2?
Reduced receptor binding
Which lipoprotein remnants accumulate in dysbetalipoproteinemia?
Chylomicron and VLDL remnants
What happens to TAG levels in dysbetalipoproteinemia?
Increased
What happens to total cholesterol in dysbetalipoproteinemia?
Increased
What happens to HDL levels in dysbetalipoproteinemia?
Decreased
What happens to LDL levels in dysbetalipoproteinemia?
Decreased
What is familial hypertriglyceridemia characterized by?
Elevated triglycerides
What is familial combined hypercholesterolemia characterized by?
Elevated cholesterol and/or triglycerides
What is the key enzyme deficient in Type I hyperlipoproteinemia?
Lipoprotein lipase
Which apolipoprotein activates lipoprotein lipase?
Apo C-II
Which apolipoprotein is essential for chylomicron formation?
Apo B-48
Which apolipoprotein mediates remnant clearance?
Apo E
Which receptor is defective in familial hypercholesterolemia?
LDL receptor
Which lipoprotein is elevated in familial hypercholesterolemia?
LDL
Which lipoprotein accumulates in chylomicron syndrome?
Chylomicrons
Which lipoproteins accumulate in dysbetalipoproteinemia?
Chylomicron and VLDL remnants
Which enzyme is high yield for cholesterol ester formation in enterocytes?
ACAT
What is the hallmark stool finding of fat malabsorption?
Steatorrhea