Lipid Metabolism and Chylomicrons Quiz

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Questions and Answers

What is the primary function of chylomicrons in the body?

  • Conversion of glucose into fatty acids
  • Synthesis of bile acids in the liver
  • Transport of dietary lipids into the blood circulation (correct)
  • Storage of lipids in adipose tissue

Where are long-chain fatty acids processed to form triacylglycerol in enterocytes?

  • Mitochondria
  • Lysosome
  • Golgi apparatus
  • Smooth endoplasmic reticulum (correct)

Which apolipoprotein is synthesized first during the formation of chylomicrons?

  • Apo B100
  • Apo A
  • Apo B48 (correct)
  • Apo C

What role do mixed micelles play in lipid absorption?

<p>Enhance the solubility of long-chain fatty acids for absorption (B)</p> Signup and view all the answers

In which system are chylomicrons initially formed after lipid digestion?

<p>Lymphatic system (C)</p> Signup and view all the answers

What happens to chylomicrons after being released from enterocytes?

<p>They enter the lymphatic system (D)</p> Signup and view all the answers

What is a clinical manifestation of abetalipoproteinemia?

<p>Formation of lipid droplets in the intestine (D)</p> Signup and view all the answers

What is the primary defect in Familial type III hyperlipoproteinemia?

<p>Deficiency in remnant clearance by the liver (C)</p> Signup and view all the answers

Which condition is primarily associated with Familial Hypertriacylglycerolemia?

<p>Hyperinsulinemia (A)</p> Signup and view all the answers

Which process occurs in the smooth endoplasmic reticulum during lipid metabolism?

<p>Esterification of fatty acids to form triacylglycerol (A)</p> Signup and view all the answers

What is the main characteristic of atherosclerosis?

<p>Buildup of lipids and cellular debris in arterial walls (C)</p> Signup and view all the answers

What is one of the consequences of hepatic lipase deficiency?

<p>Accumulation of triacylglycerol-rich lipoproteins (C)</p> Signup and view all the answers

What describes the lipid profile associated with high risk for atherosclerosis?

<p>High total cholesterol with high LDL (D)</p> Signup and view all the answers

Which lipoprotein is primarily responsible for transporting cholesterol to peripheral cells?

<p>LDL (B)</p> Signup and view all the answers

What is the main function of HDL in relation to cholesterol?

<p>Remove excess cholesterol from tissues (C)</p> Signup and view all the answers

In familial hypercholesterolemia, which receptor is primarily defective?

<p>LDL receptor (A)</p> Signup and view all the answers

What transports dietary lipids in the body?

<p>Chylomicrons (B)</p> Signup and view all the answers

Which apolipoproteins are synthesized in the liver and transferred to HDL?

<p>Apo C and Apo E (B)</p> Signup and view all the answers

How does HDL prevent thrombus formation?

<p>By stimulating prostacyclin synthesis (B)</p> Signup and view all the answers

What characterizes hyperlipoproteinemia?

<p>Elevated concentration of specific lipoprotein particles (A)</p> Signup and view all the answers

Which lipoprotein is involved in receptor-mediated endocytosis of cholesterol?

<p>LDL (B)</p> Signup and view all the answers

What is the primary structural component of VLDL and chylomicrons?

<p>Apo B (D)</p> Signup and view all the answers

Which apo protein acts as a cofactor for lipoprotein lipase?

<p>Apo C-II (A)</p> Signup and view all the answers

Which process describes the catabolism of VLDL?

<p>Progressive hydrolysis with loss of apo C (B)</p> Signup and view all the answers

Which apo protein is essential for the formation of VLDL?

<p>Apo B-100 (A)</p> Signup and view all the answers

What do the remnants of VLDL form in humans?

<p>Intermediate-density lipoprotein (IDL) (A)</p> Signup and view all the answers

Which receptor does apo B-100 primarily interact with?

<p>LDL receptor (B)</p> Signup and view all the answers

Which of the following statements about the metabolism of lipoproteins is true?

<p>Liver and intestines are key sites for lipoprotein secretion. (A)</p> Signup and view all the answers

During VLDL metabolism, what is the main result of hydrolysis by lipoprotein lipase?

<p>Loss of triacylglycerol and formation of free fatty acids (B)</p> Signup and view all the answers

What is the primary mechanism by which chylomicrons are released from the cell?

<p>Reverse pinocytosis (B)</p> Signup and view all the answers

What happens to approximately 90% of the triacylglycerol in chylomicrons?

<p>It is lost during catabolism by lipoprotein lipase (B)</p> Signup and view all the answers

What characterizes chylomicron remnants after the catabolism of chylomicrons?

<p>They contain mostly cholesteryl esters (C)</p> Signup and view all the answers

Which enzyme facilitates the uptake of chylomicron remnants by the liver?

<p>Hepatic lipase (C)</p> Signup and view all the answers

Based on density, which of the following lipoproteins has the lowest density?

<p>Chylomicrons (C)</p> Signup and view all the answers

Which apolipoprotein is predominant in Chylomicrons?

<p>Apolipoprotein B-48 (C)</p> Signup and view all the answers

What is the major component of the nonpolar lipid core of lipoproteins?

<p>Triacylglycerol (B)</p> Signup and view all the answers

Which type of lipoproteins is characterized by having a higher density than LDL?

<p>High-density lipoproteins (HDL) (D)</p> Signup and view all the answers

What type of disorder results from inherited defects in lipoprotein metabolism?

<p>Primary disorders (A)</p> Signup and view all the answers

Which of the following conditions is NOT a secondary disorder that can cause alterations in lipoproteins?

<p>Abetalipoproteinemia (D)</p> Signup and view all the answers

What is a characteristic of Tangier disease?

<p>Low levels of HDL (B)</p> Signup and view all the answers

Familial lipoprotein lipase deficiency leads to which of the following conditions?

<p>Hypertriacylglycerolemia (A)</p> Signup and view all the answers

Which of the following is a defect associated with familial alpha-lipoprotein deficiency?

<p>Low HDL levels (A)</p> Signup and view all the answers

What is the main consequence of a defective LDL receptor in familial hypercholesterolemia?

<p>Elevated LDL levels (B)</p> Signup and view all the answers

In which disorder are chylomicrons and VLDL not cleared effectively due to LPL deficiency?

<p>Familial lipoprotein lipase deficiency (D)</p> Signup and view all the answers

Which of the following best describes hyperlipoproteinemia associated with familial hypercholesterolemia?

<p>Elevated LDL levels (D)</p> Signup and view all the answers

Flashcards

What are lipoproteins?

Lipoproteins are particles composed of lipids and proteins responsible for transporting lipids throughout the body.

What is the exogenous pathway?

Exogenous pathway focuses on the absorption of dietary fats. It involves the transport of lipids from the intestine to the liver and tissues.

What is the endogenous pathway?

Endogenous pathway focuses on the synthesis and transport of lipids within the body. It involves the synthesis and transport of lipids from the liver to other tissues.

What are chylomicrons?

Chylomicrons are large lipoprotein particles primarily composed of triglycerides. They transport dietary lipids from the intestines to other tissues for energy storage or utilization.

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What are VLDL and LDL?

Very-low-density lipoproteins (VLDL) and low-density lipoproteins (LDL) are smaller lipoproteins primarily composed of triglycerides and cholesterol. They transport lipids synthesized in the liver to other tissues.

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What are HDLs?

High-density lipoproteins (HDL) are small lipoproteins primarily composed of cholesterol. They transport cholesterol back to the liver for recycling or excretion.

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What is Apolipoprotein B?

Apolipoprotein B is a protein crucial for lipoprotein assembly and function. It helps attach lipids to lipoproteins and facilitates recognition by receptors.

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What is Abetalipoproteinemia?

Abetalipoproteinemia is a rare genetic disorder that affects the formation and production of lipoproteins. This can lead to lipid accumulation in the intestines and liver.

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Chylomicrons: What are they?

Chylomicrons are triglyceride-rich lipoproteins primarily synthesized in the small intestine. They transport dietary fats (primarily triglycerides) to the rest of the body through the lymphatic system, and ultimately the bloodstream.

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How are chylomicrons transported?

Chylomicrons are released from intestinal cells into the lymphatic system via reverse pinocytosis.

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What enzyme breaks down chylomicrons?

Lipoprotein lipase (LPL) is an enzyme that breaks down triglycerides in chylomicrons, primarily in muscle and adipose tissues.

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What happens to chylomicron remnants?

After LPL action, the remnants of chylomicrons (smaller and enriched in cholesterol) are taken up by the liver via receptor-mediated endocytosis.

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How are lipoproteins classified?

Lipoproteins are classified based on their density, with chylomicrons being the least dense and HDL being the most dense.

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What is another way to classify lipoproteins?

Lipoproteins can also be classified by their electrophoretic mobility, with LDL migrating in the beta region, VLDL in the pre-beta region, IDL in the broad beta region, and HDL in the alpha region.

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What is the role of apo B in lipoproteins?

Apo B, a key structural component of VLDL and chylomicrons, plays a crucial role in the formation of lipoproteins.

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What is the function of apo C-II?

Apo C-II acts as an enzyme cofactor for lipoprotein lipase, facilitating the breakdown of triglycerides in VLDL and chylomicrons.

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What is the function of apo A-I?

Apo A-I acts as an enzyme cofactor for lecithin: cholesterol acyltransferase (LCAT), which is crucial for cholesterol transport and metabolism.

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How do apo B-100 and apo E interact with cells?

Apo B-100 and apo E act as ligands, interacting with the LDL receptor in cells, facilitating the uptake and metabolism of cholesterol.

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What is VLDL?

VLDL (Very Low Density Lipoproteins) transport triglycerides and cholesterol from the liver to other tissues.

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What is LDL?

LDL (Low Density Lipoproteins) primarily deliver cholesterol to cells. High levels of LDL are associated with an increased risk of cardiovascular disease.

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What is HDL?

HDL (High Density Lipoproteins) collect excess cholesterol from cells and transport it back to the liver for excretion. HDL is sometimes referred to as 'good cholesterol'.

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What is lipoprotein lipase?

Lipoprotein lipase, located on the endothelial surface of blood vessels, hydrolyzes triglycerides in VLDL and chylomicrons, releasing fatty acids for uptake by tissues.

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Familial Hypercholesterolemia

A genetic disorder where the LDL receptor is defective, leading to high cholesterol levels in the blood.

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Reverse Cholesterol Transport

The process of transporting cholesterol from the body's tissues back to the liver for elimination.

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HDL (High-Density Lipoprotein)

A type of lipoprotein responsible for transporting cholesterol from tissues back to the liver for recycling or excretion. Known as 'good cholesterol'.

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LDL (Low-Density Lipoprotein)

A type of lipoprotein responsible for transporting cholesterol to peripheral cells. Known as 'bad cholesterol'.

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HDL Synthesis

The process of forming new HDL particles, primarily in the liver and intestines.

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Hyperlipoproteinemia

A metabolic disorder characterized by abnormally high levels of specific lipoprotein particles in blood.

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Hyperlipidemia

A general term for elevated levels of cholesterol or triglycerides in the blood.

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Chylomicrons

A lipoprotein particle that delivers lipids from the intestines to other tissues, contributing to the 'exogenous' pathway of lipid transport.

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Familial type III hyperlipoproteinemia

A condition arising from a deficiency in chylomicron and VLDL remnant removal by the liver due to abnormal ApoE.

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What are the characteristics of familial type III hyperlipoproteinemia?

Increase in chylomicron and VLDL remnants, leading to high cholesterol, xanthomas, and atherosclerosis (broad beta disease, remnant removal disease, familial dysbetalipoproteinemia )

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Familial Hypertriacylglycerolemia (type IV)

A condition caused by overproduction of very-low-density lipoprotein (VLDL), often associated with glucose intolerance and hyperinsulinemia.

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What are the characteristics of Familial Hypertriacylglycerolemia (type IV)?

High cholesterol, VLDL (very-low-density lipoprotein), subnormal LDL (low-density lipoprotein) and HDL (high-density lipoprotein). Also associated with alcoholism, diabetes mellitus and obesity.

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Hepatic lipase deficiency

A condition caused by a deficiency of hepatic lipase, leading to the accumulation of large triacylglycerol-rich chylomicrons and VLDL remnants.

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Primary Dyslipoproteinemias

Disorders where alterations in lipoproteins occur due to genetic mutations, affecting lipoprotein metabolism.

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Secondary Dyslipoproteinemias

Disorders where alterations in lipoproteins occur due to other underlying conditions, not genetic mutations.

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Abetalipoproteinemia

A rare genetic disorder characterized by the absence of chylomicrons, VLDL, and LDL. This leads to low blood acylglycerols, and accumulation in the intestines and liver.

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Familial alpha-lipoprotein deficiency

A genetic disorder characterized by low or absent HDL, due to a deficiency in apolipoprotein C-II.

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Familial Hypercholesterolemia (type IIa)

A genetic disorder characterized by defective LDL receptors or mutations in the ligand region of apo B-100, leading to high LDL levels and hypercholesterolemia.

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Familial Lipoprotein Lipase Deficiency (type I)

A genetic disorder caused by a deficiency in lipoprotein lipase (LPL) activity, resulting in high levels of triglycerides and chylomicrons.

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Familial Lipoprotein Lipase Deficiency

A disorder characterized by slow clearance of chylomicrons and VLDL from the blood, leading to high levels of triglycerides and low levels of LDL and HDL.

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Abetalipoproteinemia

A genetic disorder affecting the formation and production of lipoproteins, resulting in lipid accumulation in the intestines and liver.

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Study Notes

Lipoprotein Metabolism

  • Lipoproteins are complexes of lipids and proteins
  • They transport lipids throughout the body
  • Triglycerides, phospholipids, cholesterol (free and bound), and protein are components

Lipoprotein Classification

  • Lipoproteins are categorized based on density
  • Types of lipoproteins include chylomicrons, VLDL, IDL, LDL, and HDL

Chylomicron Metabolism

  • Chylomicrons transport dietary lipids from the intestines
  • Synthesis includes apo B48 and apo B100
  • Lipoprotein lipase breaks down triglycerides within the chylomicrons, releasing fatty acids
  • Chylomicron remnants are taken up by the liver through receptor-mediated endocytosis
  • The liver metabolizes cholesterol and triacylglycerols in the remnants

VLDL Metabolism

  • VLDLs transport endogenous lipids (triacylglycerols) from the liver to tissues
  • They are synthesized in the liver
  • Lipoprotein lipase plays a crucial role in breaking down triglycerides in VLDL
  • VLDL remnants (IDL) are partly metabolized by the liver and partly transformed to LDL

LDL Metabolism

  • LDL transports cholesterol from the liver to peripheral tissues
  • The liver and extrahepatic tissues express LDL receptors that take up LDL via receptor-mediated endocytosis
  • Approximately 30% of LDL is degraded in extrahepatic tissues, and 70% in the liver
  • Plasma LDL cholesterol is linked to coronary atherosclerosis

HDL Metabolism

  • HDL participates in reverse cholesterol transport, removing excess cholesterol from peripheral tissues and transporting it to the liver
  • HDL is synthesized in the liver and intestines
  • HDL particles facilitate the movement of cholesteryl esters into the liver via receptor-mediated endocytosis

Apolipoproteins (Apo)

  • Apoproteins are proteins associated with lipoproteins
  • They have structural roles, act as cofactors for enzymes, and as ligands for lipoprotein receptors

Fatty Liver Disease

  • Fatty liver disease occurs when lipids accumulate in the liver
  • Blood indicators (total lipids, triglycerides, cholesterol, phospholipids) may be elevated

Lipid Absorption

  • Short-chain and medium-chain fatty acids are absorbed directly by the portal vein into the liver
  • Long-chain fatty acids are absorbed with bile salts to form micelles into the mucosal cells

Hyperlipidemia

  • Elevated plasma cholesterol or triglyceride levels or both indicate hyperlipidemia (hyperlipoproteinemia)

Atherosclerosis

  • A condition involving the buildup of lipids, cholesterol, calcium, and cellular debris within the walls of arteries (intima).
  • The accumulation leads to a narrowing of the arteries, which potentially restricts blood flow
  • Accumulation of cholesterol in artery intima is a primary step in atherosclerosis.
  • Monocytes enter the affected areas and cholesterol is endocytosed into these blood cells
  • This process leads to damage of endothelium and foam cell formation

Primary and Secondary Disorders of Lipoprotein Metabolism

  • Primary disorders are from genetic mutations impacting lipoproteins' transport or formation
  • Secondary disorders result from other systemic issues (e.g., pregnancy, hypothyroidism, or diabetes)

Lipoproteins Disorder Classification Table

  • Provides detailed characteristics and defect information for various lipoprotein disorders (e.g., abetalipoproteinemia, familial hypercholesterolemia, familial lipoprotein lipase deficiency).

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