Eukaryotic Cell Components Quiz
46 Questions
0 Views

Choose a study mode

Play Quiz
Study Flashcards
Spaced Repetition
Chat to Lesson

Podcast

Play an AI-generated podcast conversation about this lesson

Questions and Answers

In I-cell disease, the primary defect leads to the mislocalization of enzymes due to a failure in what process?

  • Calcium storage within the sarcoplasmic reticulum.
  • Phosphorylation of lysosomal enzymes in the Golgi apparatus. (correct)
  • Proper folding of proteins within the endoplasmic reticulum (ER).
  • Vesicle formation at the trans face of the Golgi.
  • Which cellular component is responsible for the synthesis of proteins destined for secretion or incorporation into the plasma membrane?

  • Golgi apparatus
  • Rough endoplasmic reticulum (RER) (correct)
  • Sarcoplasmic reticulum
  • Smooth endoplasmic reticulum (SER)
  • If newly synthesized proteins in the endoplasmic reticulum fail to achieve their correct three-dimensional structure, which cellular response is most likely to be triggered?

  • Enhanced vesicle transport from the cis face of the Golgi
  • Upregulation of calcium storage within the sarcoplasmic reticulum
  • Activation of the unfolded protein response (UPR) (correct)
  • Increased protein synthesis to compensate
  • A cell with a mutation affecting the function of GlcNac-1-phosphotransferase would most directly exhibit which of the following?

    <p>A build-up of lysosomal enzymes outside of the lysosome. (B)</p> Signup and view all the answers

    What is the primary function of the sarcoplasmic reticulum in muscle cells?

    <p>Storing intracellular calcium (C)</p> Signup and view all the answers

    A patient exhibits premature aging affecting the musculoskeletal and cardiovascular systems. Which organelle is most likely implicated?

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

    Which of the following is associated with the accumulation of gangliosides in neurons, leading to neurological degeneration and a cherry-red spot?

    <p>Tay-Sachs disease (A)</p> Signup and view all the answers

    A defect in which organelle is most likely to cause a buildup of very long chain fatty acids in the blood?

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

    A patient with neuromuscular disease symptoms most likely has a defect in which organelle?

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

    Skeletal muscle abnormalities, mental defects and an accumulation of GAGs in the urine are most closely associated with a defect in which enzyme?

    <p>α-L-iduronidase (C)</p> Signup and view all the answers

    Which of the following is characterized by defective physical growth and mental defects, along with secreted lysosomal enzymes present in the blood?

    <p>I-cell disease (A)</p> Signup and view all the answers

    Impaired brain development, and lesions in the liver and kidneys are most likely associated with a defect in which organelle?

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

    A five year old girl presents to her physician with delays in physical growth, and inability to form full sentences. Lab results show the presence of polysaccharides in her urine. What is most likely defected?

    <p>Alpha-L-iduronidase (B)</p> Signup and view all the answers

    A patient presents with symptoms including premature aging, disfigured nuclei, and cardiovascular issues. Which protein is most likely mutated in this patient?

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

    A deficiency in which enzyme causes a buildup of ganglioside in neurons, leading to neurological deterioration and a characteristic cherry-red spot on the retina?

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

    A buildup of glycosaminoglycans (GAGs) in various tissues, along with skeletal abnormalities and mental defects, is characteristic of a deficiency in which enzyme?

    <p>Alpha-L-iduronidase (C)</p> Signup and view all the answers

    A mutation in peroxins would directly lead to dysfunction in which process?

    <p>Beta oxidation of fatty acids (C)</p> Signup and view all the answers

    A patient has a build-up of very long chain fatty acids in their blood. Which organelle is most likely not functioning correctly?

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

    Mitochondrial myopathies are often difficult to diagnose. Which of the following is a reason for this difficulty?

    <p>Symptoms vary widely depending on which organs are affected. (B)</p> Signup and view all the answers

    What is the primary function of the enzyme catalase found within peroxisomes?

    <p>To convert H2O2 to H2O and O2. (D)</p> Signup and view all the answers

    A cell’s nuclear lamina provides structural support that helps provide shape and rigidity to which of the following?

    <p>The inner nuclear membrane (A)</p> Signup and view all the answers

    What is the name of the truncated protein that results from a mutation in Lamin A, implicated in the disease progeria?

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

    Which organelle is correctly matched with its clinical correlate?

    <p>Lysosomes: Hurler Syndrome (A)</p> Signup and view all the answers

    Match the following diseases with the organelle that is primarily affected.

    <p>Tay-Sachs Disease = Lysosomes Zellweger Syndrome = Peroxisomes Progeria = Nucleus Mitochondrial Myopathies = Mitochondria</p> Signup and view all the answers

    Match the following enzymes with the disease in which they are deficient.

    <p>Hexosaminidase A (Hex A) = Tay-Sachs Disease alpha-L-iduronidase = Hurler Syndrome Catalase = Zellweger Syndrome Lamin A = Progeria</p> Signup and view all the answers

    Match the following disease symptoms with the corresponding organelle.

    <p>Accumulation of very long chain fatty acids in the blood = Peroxisomes Cherry-red spot on the retina = Lysosomes Premature aging, disfigured nuclei = Nucleus Weakness, drooping eyelids, swallowing difficulties = Mitochondria</p> Signup and view all the answers

    Match the following diseases with the primary defect that causes them.

    <p>Tay-Sachs Disease = Deficiency in a lysosomal enzyme Zellweger Syndrome = Mutation in peroxins Progeria = Mutation in Lamin A Mitochondrial Myopathies = High mutation rate in mtDNA</p> Signup and view all the answers

    Match the following conditions with the organelle most directly impacted.

    <p>Accumulation of gangliosides in neurons = Lysosomes Defective brain development and impaired fatty acid metabolism = Peroxisomes Premature aging and nuclear envelope abnormalities = Nucleus Skeletal muscle weakness and dysfunction of oxidative phosphorylation = Mitochondria</p> Signup and view all the answers

    Match the following diseases with the characteristic clinical features.

    <p>Tay-Sachs Disease = Neurological degeneration, cherry-red spot on retina Zellweger Syndrome = Impaired brain development, accumulation of very long chain fatty acids Progeria = Premature aging, disfigured nuclei, cardiovascular issues Mitochondrial Myopathies = Variable weakness, often affecting skeletal muscles</p> Signup and view all the answers

    Match the following diseases with the accumulation of specific substances in the body.

    <p>Tay-Sachs Disease = Gangliosides Zellweger Syndrome = Very long chain fatty acids Hurler Syndrome = Glycosaminoglycans None of the above = Progeria</p> Signup and view all the answers

    Match the following diseases with the affected organ system.

    <p>Tay-Sachs Disease = Nervous system Zellweger Syndrome = Brain, liver, kidneys Progeria = Musculoskeletal and cardiovascular systems Mitochondrial Myopathies = Musculoskeletal and other systems</p> Signup and view all the answers

    Match the following diseases with the inheritance pattern.

    <p>Tay-Sachs Disease = Autosomal recessive Zellweger Syndrome = Autosomal recessive Progeria = Autosomal dominant Mitochondrial Myopathies = Mitochondrial inheritance</p> Signup and view all the answers

    Match the following diseases with the typical age of symptom onset.

    <p>Tay-Sachs Disease = Infancy Zellweger Syndrome = Infancy Progeria = Childhood Mitochondrial Myopathies = Variable, can be present at birth or later</p> Signup and view all the answers

    Match the following diseases with their associated defective organelles:

    <p>I-cell disease = Endoplasmic reticulum Zellweger syndrome = Peroxisome Tay-Sachs disease = Lysosome Mennell syndrome = Mitochondria</p> Signup and view all the answers

    Match the following clinical features with the corresponding disease:

    <p>I-cell disease = Build-up of lysosomal enzymes in blood Zellweger syndrome = Impaired brain development Tay-Sachs disease = Cherry-red spot on retina Mennell syndrome = Premature aging and cardiovascular issues</p> Signup and view all the answers

    Match the following defects with their enzymatic consequences:

    <p>I-cell disease = Non-phosphorylation of lysosomal enzymes Gaucher disease = Deficiency of glucocerebrosidase Krabbe disease = Deficiency of galactocerebrosidase Fabry disease = Deficiency of alpha-galactosidase</p> Signup and view all the answers

    Match the organelle with its impact when defective:

    <p>Endoplasmic reticulum = Failure in protein synthesis Golgi apparatus = Improper sorting and packaging of proteins Lysosome = Accumulation of waste material Peroxisome = Build-up of very long chain fatty acids</p> Signup and view all the answers

    Match the organelle dysfunction with the potential disease symptoms:

    <p>Endoplasmic reticulum = Defective growth and mental defects Peroxisome = Neurological degeneration Golgi apparatus = Excess secretory vesicles in blood Lysosome = A buildup of glycosaminoglycans in urine</p> Signup and view all the answers

    Match the following diseases with their associated organelles:

    <p>Progeria = Nucleus Mitochondrial Myopathies = Mitochondria Tay-Sachs = Lysosomes Zellweger = Peroxisomes</p> Signup and view all the answers

    Match the diseases with their primary mutations:

    <p>Progeria = Lamin A Tay-Sachs = Hex A I cell disease = GlcNac-1-phosphotransferase Hurler Syndrome = α-L-iduronidase</p> Signup and view all the answers

    Match the diseases with their notable features:

    <p>Mitochondrial Myopathies = Neuromuscular disease symptoms Hurler Syndrome = Skeletal muscle abnormalities, mental defects Zellweger = Impaired brain development, liver and kidney lesions I cell disease = Defective physical growth and mental defects</p> Signup and view all the answers

    Match the organelles with their associated defects:

    <p>Nucleus = Lamin A mutation Mitochondria = mtDNA or nuclear DNA mutation Lysosomes = Hex A deficiency Peroxisomes = Peroxins deficiency</p> Signup and view all the answers

    Match the diseases with their clinical manifestations:

    <p>Tay-Sachs = Cherry red spot Progeria = Premature aging of the MSK and CV systems Zellweger = Very long chain fatty acids in blood I cell disease = Secreted lysosomal enzymes in blood</p> Signup and view all the answers

    Match the diseases with the type of accumulation:

    <p>Tay-Sachs = Ganglioside Hurler Syndrome = Glycosaminoglycans (GAGs) Progeria = Truncated Lamin A Zellweger = Very long chain fatty acids</p> Signup and view all the answers

    Match the diseases with their diagnostic methods:

    <p>Progeria = Familial history and clinical features Tay-Sachs = Enzyme assay for Hex A Hurler Syndrome = Urine GAGs analysis Zellweger = Blood fatty acid profile</p> Signup and view all the answers

    Match the organelles with their corresponding diseases based on enzyme deficiencies:

    <p>Lysosomes = Tay-Sachs Nucleus = Progeria Peroxisomes = Zellweger Golgi Apparatus = I cell disease</p> Signup and view all the answers

    Flashcards

    Endoplasmic Reticulum (ER)

    A network of interconnected sacs and tubules throughout the cytoplasm of eukaryotic cells. Plays a crucial role in the synthesis, folding, and modification of proteins.

    Sarcoplasmic Reticulum

    A specialized type of endoplasmic reticulum found in muscle cells. It serves as a storage site for intracellular calcium, essential for muscle contraction.

    Unfolded Protein Response (UPR)

    A cellular quality control mechanism that ensures proper folding of newly synthesized proteins. If a protein misfolds, the ER triggers a response that can lead to cell death.

    Golgi Apparatus

    A stack of flattened membrane-bound sacs involved in the modification, packaging, and sorting of proteins and lipids. It functions like a 'post office' for the cell.

    Signup and view all the flashcards

    I-Cell Disease

    A rare genetic disorder caused by a deficiency in an enzyme responsible for phosphorylating lysosomal enzymes. This leads to the accumulation of undigested material within cells, resulting in severe developmental and health issues.

    Signup and view all the flashcards

    Progeria

    A rare genetic disorder characterized by premature aging, primarily affecting the musculoskeletal and cardiovascular systems.

    Signup and view all the flashcards

    Mitochondrial Myopathies

    A group of disorders caused by mutations in genes that affect mitochondrial function.

    Signup and view all the flashcards

    Tay-Sachs

    A rare genetic disorder characterized by progressive neurological degeneration due to the accumulation of gangliosides in neurons.

    Signup and view all the flashcards

    Hurler Syndrome

    A severe genetic disorder caused by a deficiency in the enzyme alpha-L-iduronidase, leading to the accumulation of glycosaminoglycans (GAGs) in various tissues.

    Signup and view all the flashcards

    Tay-Sachs Disease

    A genetic disorder caused by a deficiency in the enzyme hexosaminidase A (Hex A), leading to the accumulation of gangliosides in neurons.

    Signup and view all the flashcards

    Zellweger Syndrome

    A group of genetic disorders affecting the peroxisomes, organelles responsible for various metabolic processes.

    Signup and view all the flashcards

    Hurler Syndrome

    A rare genetic disorder characterized by defective physical growth and mental development, caused by a deficiency in the enzyme alpha-L-iduronidase.

    Signup and view all the flashcards

    Na+/K+ ATPase (Sodium-Potassium Pump)

    A transmembrane protein that pumps three sodium ions (Na+) out of the cell and two potassium ions (K+) into the cell. This process requires energy obtained from ATP hydrolysis and is crucial for maintaining cell membrane potential.

    Signup and view all the flashcards

    Nucleus

    The control center of the cell that stores the genetic material, DNA, wrapped in a double-layered membrane called the nuclear envelope.

    Signup and view all the flashcards

    Smooth Endoplasmic Reticulum (SER)

    A specialized type of ER lacking ribosomes, responsible for synthesizing lipids, steroids, and detoxifying harmful substances.

    Signup and view all the flashcards

    Mitochondria

    The powerhouse of the cell, responsible for generating ATP through cellular respiration and oxidative phosphorylation.

    Signup and view all the flashcards

    Lysosome

    A tiny, membrane-enclosed organelle containing digestive enzymes (hydrolases) that break down macromolecules.

    Signup and view all the flashcards

    Peroxisome

    Small, membrane-bound organelles that play a critical role in detoxification, particularly breakdown of very long fatty acids.

    Signup and view all the flashcards

    What is the role of RER?

    RER (Rough Endoplasmic Reticulum) is covered in ribosomes, where secretory proteins are synthesized. Secretory proteins are those that will be transported to the plasma membrane or incorporated into vesicles.

    Signup and view all the flashcards

    What is the function of the sarcoplasmic reticulum?

    The sarcoplasmic reticulum (SR) is a specialized type of ER found in muscle cells. It stores intracellular calcium, which is essential for muscle contraction.

    Signup and view all the flashcards

    What is the Unfolded Protein Response (UPR)?

    The Unfolded Protein Response (UPR) is a quality control mechanism ensuring newly synthesized proteins fold correctly. If proteins misfold, the ER can shut down protein synthesis to prevent problems, and the cell may undergo apoptosis.

    Signup and view all the flashcards

    Describe the functions of the Golgi apparatus.

    The Golgi apparatus is a stack of flattened membrane-bound sacs that modify, package, and sort proteins and lipids. It receives transport vesicles from the ER on its cis face and releases secretory vesicles from its trans face.

    Signup and view all the flashcards

    What is I-cell disease?

    I-cell disease is a genetic disorder caused by a deficiency in an enzyme that normally phosphorylates lysosomal enzymes. This leads to unphosphorylated enzymes being released from the cell, which means the lysosomes lack the necessary enzymes to function properly.

    Signup and view all the flashcards

    What does the Na+/K+ ATPase do?

    Pumps 3 sodium ions (Na+) out of the cell and 2 potassium ions (K+) into the cell, using energy from ATP hydrolysis. This creates an electrochemical gradient across the cell membrane, crucial for nerve impulse transmission and maintaining cell volume.

    Signup and view all the flashcards

    What is the function of the nucleus?

    The cell's control center containing DNA, surrounded by a double-layered membrane called the nuclear envelope. It regulates gene expression and protein synthesis.

    Signup and view all the flashcards

    What is the role of the nuclear lamina?

    The inner nuclear membrane (INM) is lined by a protein framework called the nuclear lamina, which provides structural support and shape to the nucleus.

    Signup and view all the flashcards

    How is the outer nuclear membrane related to the ER?

    The outer nuclear membrane (ONM) is continuous with the endoplasmic reticulum (ER) membrane, suggesting a connection between these two cellular structures.

    Signup and view all the flashcards

    What is Progeria?

    A rare genetic disorder caused by a mutation in the gene for lamin A, a key protein in the nuclear lamina. This leads to a truncated protein called progerin, which disrupts nuclear structure and causes premature aging.

    Signup and view all the flashcards

    What is the function of mitochondria?

    The powerhouse of the cell, responsible for generating ATP through oxidative phosphorylation. It has a double membrane structure with an inner membrane containing the electron transport chain and an outer membrane surrounding the matrix.

    Signup and view all the flashcards

    How is mtDNA inherited?

    Mitochondrial DNA (mtDNA) is inherited solely from the mother and plays a role in energy production within mitochondria.

    Signup and view all the flashcards

    What are Mitochondrial Myopathies?

    A group of disorders caused by mutations in genes involved in mitochondrial function. Symptoms vary but generally involve muscle weakness due to impaired energy production in muscle cells.

    Signup and view all the flashcards

    What is the function of lysosomes?

    A membrane-bound organelle containing digestive enzymes called hydrolases. It breaks down macromolecules, plays a role in immunity by destroying engulfed pathogens, and recycles cellular components.

    Signup and view all the flashcards

    What is Tay-Sachs Disease?

    A rare genetic disorder caused by a deficiency in the enzyme hexosaminidase A (Hex A), leading to the accumulation of gangliosides in neurons. This causes progressive neurological deterioration, leading to death by age 4.

    Signup and view all the flashcards

    Study Notes

    Eukaryotic Cell Components

    • Plasma membrane

      • Selectively permeable to ions and organic molecules
      • Phospholipid bilayer (2 hydrophobic fatty acid tails connected to a hydrophilic head)
      • Major component: Lipids + Proteins
        • Lipids
          • 75% phospholipids
          • 20% cholesterol
          • 5% glycolipids
          • Contribute to membrane fluidity
          • Cell recognition (ABO blood group antigens)
        • Proteins
          • Integral membrane proteins (permanent association)
          • Peripheral membrane proteins (transient association)
          • Involved in transporting, anchoring, and signaling
          • Examples: channels, receptors, pumps
    • 6 membrane-enclosed organelles

      • Nucleus

        • Information storage (DNA)
        • Surrounded by a nuclear envelope with 2 lipid bilayers
        • Inner nuclear membrane (INM) proteins form nuclear lamina (shapes and structural integrity)
        • Outer nuclear membrane (ONM) is continuous with ER membrane
        • Progeria case study: mutation in Lamin A leads to a truncated protein, progerin, causing premature aging.
      • Mitochondria

        • Membrane-bound organelle (inner and outer membrane with matrix inside)
        • Functions include energy production (ATP), oxidative phosphorylation, Krebs cycle, fatty acid and steroid synthesis.
        • Mitochondrial DNA (mtDNA) originates solely from the mother, encoding for many proteins required by the mitochondria
        • Mitochondrial Myopathies: mutations in mtDNA can cause various neuromuscular weaknesses, including drooping eyelids and swallowing difficulties., symptoms depend upon which organs are affected.
      • Lysosomes

        • Membrane-bound organelles filled with digestive enzymes (hydrolases)
        • Break down macromolecules.
        • Important in immunity by breaking down engulfed pathogens.
        • Tay-Sachs Disease: a deficiency in Hexosaminidase A causes ganglioside accumulation, resulting in neurological deterioration, cherry red spot on retina.
        • Hurler Syndrome: a deficiency in alpha-L-iduronidase leads to glycosaminoglycan accumulation. Skeletal muscle abnormalities and mental defects also arise.
      • Peroxisomes

        • Membrane-bound organelles
        • Provide environment for oxidative reactions (e.g., beta oxidation of very long-chain fatty acids)
        • Contains catalase, which converts hydrogen peroxide to water and oxygen.
        • Zellweger Syndrome: peroxisome biogenesis failure causes defects, fatty acid accumulation, impaired brain development, and early death.
      • Endoplasmic Reticulum (ER)

        • Membrane-bound sacs/tubules continuous with the nuclear membrane
        • Rough ER (RER) is studded with ribosomes, responsible for protein synthesis (secretory proteins) targeted toward PM or incorporation into vesicles
        • Smooth ER (SER) is continuous with RER with no ribosomes associated. A major site of new membrane synthesis and storage for intracellular calcium.
        • Unfolded protein response (UPR): Quality control for newly synthesized proteins; If misfolded, the cell tries to repair the protein or undergoes apoptosis.
      • Golgi Apparatus

        • Source of secretory vesicles, with a cis and trans face
        • Modifies, packages, and sorts proteins, notably by adding phosphate groups to certain proteins enabling their proper targeting.
        • I-cell disease: deficiency in an enzyme that modifies lysosomal enzymes, leading to defective lysosomes functioning. Lysosomal enzymes are lacking the necessary enzymes and are released directly from the cell causing a build up of these in the blood.
    • Cytoskeleton

      • Structural framework of the cell, composed of various proteins

    Ion Concentrations Inside and Outside the Cell:

    • Sodium-Potassium Pump (Na+/K+ ATPase):
    • Pumps 3 sodium ions (Na+) out of the cell and 2 potassium ions (K+) into the cell. This creates a concentration gradient.
    • 3 sodium ions out and 2 potassium ions in. This creates an important gradient for cell function.

    Studying That Suits You

    Use AI to generate personalized quizzes and flashcards to suit your learning preferences.

    Quiz Team

    Related Documents

    Eukaryotic Cell Components PDF

    Description

    Test your knowledge on the various components of eukaryotic cells, including the plasma membrane and organelles. This quiz covers the structure, function, and significance of key cellular structures such as lipids, proteins, and the nucleus. Perfect for biology students looking to reinforce their understanding of cell biology.

    More Like This

    Cell Membranes and Organelles in Eukaryotic Cells
    10 questions
    Cell Biology: Membrane Organelles
    8 questions
    Eukaryotic Cell Compartmentalization
    27 questions

    Eukaryotic Cell Compartmentalization

    AttractiveRomanticism1652 avatar
    AttractiveRomanticism1652
    Use Quizgecko on...
    Browser
    Browser