Hyperlipoproteinemia Overview
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Hyperlipoproteinemia Overview

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Questions and Answers

What is the consequence of a deficiency in lipoprotein lipase (LPL) in Hyperlipoproteinemia Type I?

  • Increased levels of cholesterol and triglycerides with no effects on HDL
  • Increased levels of HDL and reduced levels of LDL
  • Decreased levels of triglycerides and increased coronary disease risk
  • Increased levels of chylomicrons and VLDL, with decreased LDL and HDL (correct)

    • What is the primary defective component in familial hypercholesterolemia (Type IIa)?

  • Lipoprotein lipase (LPL)
  • LDL receptor (correct)
  • ApoCII
  • Triglycerides

    • Which lipid is primarily increased in patients with Type IIa hyperlipoproteinemia?

  • Chylomicrons
  • HDL
  • LDL (correct)
  • Triglycerides

    • Which therapeutic target is commonly employed to treat dyslipidemia?

    <p>Inhibition of HMG CoA reductase</p> Signup and view all the answers

    What common condition is associated with an increased incidence of atherosclerosis?

    <p>Obesity and diabetes</p> Signup and view all the answers

    What genetic defect is associated with Tangier disease?

    <p>Defect in ABCA1 transporter</p> Signup and view all the answers

    What is a primary effect of abetalipoproteinemia on lipoproteins?

    <p>Decreased levels of VLDL, IDL, and LDL</p> Signup and view all the answers

    Which lipoprotein is primarily affected by accelerated catabolism of ApoA-I and ApoA-II?

    <p>HDL</p> Signup and view all the answers

    What is the consequence of defective lysosomal acid lipases in Wolman disease?

    <p>Accumulation of cholesterol esters and TGs</p> Signup and view all the answers

    What role does LCAT play in HDL metabolism?

    <p>Converting cholesterol to cholesterol esters</p> Signup and view all the answers

    Which of the following lipoproteins is synthesized mainly in the liver and intestine?

    <p>HDL</p> Signup and view all the answers

    What is a potential outcome of LCAT deficiency?

    <p>Decreased HDL levels</p> Signup and view all the answers

    Which of the following is NOT a function of ApoA-I?

    <p>Transporting triglycerides</p> Signup and view all the answers

    What is the consequence of PCSK9 inhibition on LDL levels?

    <p>LDL levels decrease due to enhanced receptor activity</p> Signup and view all the answers

    Which lipoprotein is rich in cholesterol?

    <p>LDL</p> Signup and view all the answers

    What role does the ApoB100 play in cholesterol metabolism?

    <p>Promotes the uptake of LDL into peripheral tissues</p> Signup and view all the answers

    What is a characteristic feature of familial hypertriacylglycerolemia (Type IV hyperlipoproteinemia)?

    <p>Overproduction of VLDL associated with glucose intolerance</p> Signup and view all the answers

    What occurs as a result of defective ApoE in familial Type III hyperlipoproteinemia?

    <p>Increased levels of chylomicrons and VLDL</p> Signup and view all the answers

    What is the effect of Tangier disease on HDL levels?

    <p>Decrease in HDL levels</p> Signup and view all the answers

    What happens to LDL receptor levels with the use of PCSK9 inhibitors?

    <p>Increase in LDL receptor levels</p> Signup and view all the answers

    What is characterized by accelerated catabolism of ApoA-I and ApoA-II?

    <p>Hypoalphalipoproteinemia</p> Signup and view all the answers

    What is the primary consequence of inadequate energy or amino acids in the liver concerning VLDL synthesis?

    <p>Accumulation of triglycerides in the liver</p> Signup and view all the answers

    Which of the following interventions can help in controlling serum cholesterol levels?

    <p>Weight reduction and exercise</p> Signup and view all the answers

    What role do raised levels of HDL play in cardiovascular health?

    <p>They provide cardio-protective effects.</p> Signup and view all the answers

    Which drug is known to inhibit cholesterol absorption from the diet?

    <p>Ezetimibe</p> Signup and view all the answers

    What is associated with the development of atherosclerosis?

    <p>High levels of LDL cholesterol</p> Signup and view all the answers

    Which type of medication is associated with increasing HDL levels?

    <p>Fibrates</p> Signup and view all the answers

    The metabolic syndrome includes conditions that increase the risk for which diseases?

    <p>Cardiovascular disease and diabetes mellitus</p> Signup and view all the answers

    What is a key function of PCSK9 inhibitors in cholesterol management?

    <p>Promote LDL receptor degradation</p> Signup and view all the answers

    Study Notes

    Hyperlipoproteinemia

    • Hyperlipoproteinemia is a condition characterized by high levels of lipids in the blood.
    • Familial lipoprotein lipase (LPL) deficiency is characterized by high levels of chylomicrons and VLDL, and low levels of LDL and HDL.
    • Familial hypercholesterolemia is characterized by high levels of LDL and cholesterol.
    • Familial hypertriacylglycerolemia is characterized by high levels of VLDL and TG.
    • In familial hypercholesterolemia, defects in LDL receptor, ApoB100, and PCSK9 lead to high levels of LDL.
    • LDL (low-density lipoprotein) is normally taken up by liver or peripheral tissues via ApoB100 binding to LDL receptor, which is regulated by PCSK9.
    • PCSK9 (proprotein convertase subtilisin/kexin type 9) inhibitors, such as Alirocumab and Evolocumab, are human monoclonal antibodies that inhibit the degradation of LDLR in hepatocytes and thus enhance the uptake and clearance of circulating LDL particles.
    • Familial Type III hyperlipoproteinemia is characterized by high levels of chylomicrons and VLDL, and is often associated with ApoE deficiency.

    Hypolipoproteinemia

    • Hypolipoproteinemia is characterized by low levels of lipids in the blood.
    • Tangier disease is characterized by low levels of HDL, and is caused by defects in ABCA1, the transporter that supports cholesterol pickup by nascent HDLs.
    • Abetalipoproteinemia is characterized by low levels of chylomicrons, VLDL, IDL, and LDL, and is caused by mutations in the MTTP (microsomal triglyceride transfer protein) gene.
    • Hypoalphalipoproteinemia is characterized by low levels of HDL, and is caused by accelerated catabolism of ApoA-I and ApoA-II.

    Other Conditions

    • Wolman disease is a lysosomal storage disorder caused by a deficiency in lysosomal acid lipases.
    • LCAT (lecithin-cholesterol acyltransferase) deficiency is characterized by low HDL levels and tissue accumulation of cholesterol, often resulting in anemia, corneal opacification, and renal failure.
    • Fatty liver is a condition where excess TG accumulates in the liver.

    Atherosclerosis

    • Atherosclerosis is a disease in which plaque builds up inside the arteries.
    • The intima is the innermost layer of the arterial wall.
    • Atherosclerotic plaques develop over time, and can lead to narrowing of the arteries, which can increase the risk of heart attack and stroke.

    Treatment

    • Lifestyle/diet modifications can help lower serum cholesterol levels.
    • Statins, which inhibit HMG-CoA reductase, are commonly used to lower serum cholesterol levels.
    • PCSK9 inhibitors are used to slow LDL receptor degradation and lower LDL levels.
    • Bile acid sequestrants, such as Colestipol and Cholestyramine, increase bile acid loss in feces.
    • Nicotinic acid (niacin) is a vitamin B3, which reduces Lp(a) as well as LDL-cholesterol and TAGs, and raises HDL levels.
    • Fibrates, such as Gemfibrizol and Clofibrate, inhibit the hepatic release of lipoproteins (particularly VLDL), potentiate the action of lipoprotein lipase (LPL), and increase HDL.
    • Ezetimibe (Zetia) inhibits dietary absorption of cholesterol.

    Metabolic Syndrome

    • Metabolic syndrome is a cluster of metabolic abnormalities that increase risk of cardiovascular disease (CVD) and diabetes mellitus.

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    Lipid Metabolism Disorders Lecture - D. Zhang, NYIT

    Description

    This quiz covers the various types of hyperlipoproteinemia, including familial lipoprotein lipase deficiency and familial hypercholesterolemia. Understand the mechanisms behind lipid levels in the blood and the role of PCSK9 in LDL regulation. Test your knowledge on this important aspect of lipid metabolism.

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