Biochemistry: Sphingolipid Metabolism

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Questions and Answers

What role do sphingolipids play in intercellular communication?

  • They do not have any significant roles in cell signaling.
  • They act solely as structural components of cell membranes.
  • They facilitate the transmission of signals between cells. (correct)
  • They serve as a source of energy for cellular processes.

Which of the following is NOT a function of ceramide?

  • Serving as a component of cell membranes.
  • Acting as a signaling molecule.
  • Providing structural support to the cytoskeleton. (correct)
  • Being a receptor for bacterial toxins.

Which type of sphingolipid is characterized by having a long chain fatty acid attached via an amide linkage?

  • Phosphatidylserine
  • Glycosphingolipids
  • Sphingomyelin
  • Ceramide (correct)

What distinguishes acidic glycosphingolipids from their neutral counterparts?

<p>The charge on their surface at physiological pH. (C)</p> Signup and view all the answers

Which clinical condition is associated with sphingolipidoses?

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

What is the backbone structure of sphingolipids?

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

Which of the following diseases is specifically linked to sphingolipid metabolism issues?

<p>Gaucher’s Disease (B)</p> Signup and view all the answers

What type of interaction can sphingolipids have with viral receptors?

<p>They serve as receptors for certain viruses. (B)</p> Signup and view all the answers

What is a potential clinical implication of altered sphingolipid metabolism?

<p>Development of neurodegenerative conditions. (A)</p> Signup and view all the answers

What is the primary enzymatic activity associated with type B sphingomyelin production?

<p>Acylation at the amino group (B)</p> Signup and view all the answers

Which aspect of sphingomyelin is significant in Niemann-Pick Disease?

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

What role do ceramide and sphingosine serve as in cellular processes?

<p>Intracellular messengers and regulators (D)</p> Signup and view all the answers

What is the main function of sphingomyelinase in the body?

<p>Converting sphingomyelin to ceramide (D)</p> Signup and view all the answers

Which of the following structures differs from sphingomyelin in terms of phosphate content?

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

What role do glycolipids primarily play within cellular membranes?

<p>Acting as cell surface receptors (A)</p> Signup and view all the answers

Which statement accurately describes sphingomyelin?

<p>It serves as a major component of membranes in neural tissues. (C)</p> Signup and view all the answers

What is the primary source material for sphingomyelin synthesis?

<p>Ceramide, cytidine diphosphate, and choline (C)</p> Signup and view all the answers

What is a byproduct of the hydrolysis of sphingomyelin?

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

Which function is NOT associated with sphingomyelin?

<p>Storage of energy in adipose tissue (C)</p> Signup and view all the answers

What distinguishes sphingomyelin from other sphingolipids?

<p>The presence of a phosphate group (A)</p> Signup and view all the answers

Which acid is not yielded from the hydrolysis of sphingomyelin?

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

What role does ceramide play in sphingomyelin metabolism?

<p>It is the precursor of sphingomyelin. (C)</p> Signup and view all the answers

Which of the following is NOT a component of the structure of sphingomyelin?

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

Which one of the following is a significant characteristic of sphingomyelin in the human body?

<p>It is a key component of the myelin sheath. (C)</p> Signup and view all the answers

Which of the following statements about fat and lipid metabolism is correct?

<p>Sphingomyelin is crucial for the integrity of nerve cell membranes. (D)</p> Signup and view all the answers

What type of linkage connects a fatty acid to the amino group of sphingosine?

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

Which fatty acid is most commonly found in sphingolipid structures?

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

From which precursor compound is sphingosine synthesized?

<p>Serine and palmitoyl CoA (D)</p> Signup and view all the answers

What is the primary function of sphingomyelin in cellular structures?

<p>Cell membrane stability (A)</p> Signup and view all the answers

What is the significance of the D-erythro form of sphingosine?

<p>It is the naturally occurring form. (B)</p> Signup and view all the answers

In which human tissue are sphingolipids most concentrated?

<p>Central nervous system (CNS) (B)</p> Signup and view all the answers

What role does NADPH play in the synthesis of sphingosine?

<p>It acts as a reducing agent. (C)</p> Signup and view all the answers

What is the backbone of sphingolipids?

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

What happens to palmitoyl CoA during sphingosine synthesis?

<p>It loses CoA and reacts with ß-aminoalcohol. (A)</p> Signup and view all the answers

Which of the following is a characteristic of sphingolipids?

<p>They include sphingomyelin which is associated with phosphorylcholine. (A)</p> Signup and view all the answers

What structural feature characterizes the myelin sheath?

<p>It has a layered membranous structure. (D)</p> Signup and view all the answers

Which fatty acid does NOT contribute to myelin sheath composition?

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

What is one of the primary roles of sphingolipids synthesized from sphingosine?

<p>Maintain skin's water permeability. (B)</p> Signup and view all the answers

Which of the following describes the oxidation process involving palmitoyl CoA?

<p>It is primarily catalyzed by FAD. (B)</p> Signup and view all the answers

What characteristic defines sphingosine as a precursor for sphingolipids?

<p>It possesses two asymmetric carbon atoms. (B)</p> Signup and view all the answers

Which of the following statements about ceramide is accurate?

<p>It can form an amide bond with fatty acids of 30 carbons. (D)</p> Signup and view all the answers

Which fatty acid is specifically noted for being 22 carbons long?

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

What is a key function of myelin in the central nervous system?

<p>It isolates neuronal fibers. (A)</p> Signup and view all the answers

Which component is specifically involved in the condensation reaction with serine during myelin formation?

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

What is the primary component of the grey matter in the brain?

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

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Study Notes

Overview of Sphingolipid Metabolism

  • Functions include intercellular communication and serving as antigenic determinants for the ABO blood group.
  • Certain sphingolipids act as receptors for viruses and bacterial toxins.

Sphingolipids

  • Comprised mainly of sphingophospholipids (like sphingomyelin) and glycosphingolipids (neutral and acidic).
  • Sphingophospholipids are notable for containing a phosphate group, while glycosphingolipids contain carbohydrates.

Sphingomyelin

  • Formed by esterification of sphingosine's alcohol group at carbon 1 to phosphorylcholine.
  • Significant constituent in human membranes, especially in neural tissues and myelin sheaths.
  • Hydrolysis products include fatty acid, phosphoric acid, choline, and sphingosine.

Ceramide

  • Synthesized in the endoplasmic reticulum from serine and palmitoyl CoA; precursor to glycolipids.
  • Composed of a very-long-chain fatty acid (22 carbons) linked to sphingosine via an amide bond.
  • Functions as a structural lipid in nerve tissues and regulates skin water permeability.

Sphingosine

  • Amino alcohol with two asymmetric carbon atoms, a key precursor to sphingolipids.
  • The D-erythro form is the naturally occurring variant; synthesized from serine and palmitoyl CoA using NADPH.
  • Common fatty acid contributions include palmitic, stearic, lignoceric, and nervonic acids.

Glycosphingolipids

  • Found in all human tissues, with the highest concentrations in CNS (especially white matter).
  • Primarily derivatives of ceramide, combining lipid and carbohydrate components.
  • Carbohydrate portions exposed on the outer layer of cell membranes, acting as receptors for toxins and antigens.

Degradation

  • Sphingomyelinase and ceramidase play critical roles in the breakdown of sphingomyelin and ceramide, respectively.
  • Sphingomyelinase acts as a lysosomal enzyme that hydrolyzes sphingomyelin, yielding ceramide.

Clinical Applications

  • Sphingolipidoses, including Niemann-Pick disease, result from enzyme deficiencies affecting sphingolipid metabolism.
  • Niemann-Pick disease results in an inability to degrade sphingomyelin due to sphingomyelinase deficiency, leading to organ damage.

General Function of Sphingolipids

  • Serve as key signaling molecules and structural components in cellular membranes.
  • Regulate cellular activities like growth, development, and apoptosis through interactions with signaling pathways and other cellular receptors.

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