When analyzing a sample of cells from a patient, you find the lysosomes are filled with undigested material. This observation makes you think that the lysosomes are not functioning properly. What are three different defects that could be responsible for malfunctioning lysosomes?

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1. Enzyme Deficiency: Lysosomes contain enzymes that break down waste materials and cellular debris. If these enzymes are deficient or not functioning properly, the lysosomes will not be able to digest the material. This could be due to a genetic disorder, such as a lysosomal storage disease, where the body doesn't produce enough of a specific enzyme.

2. Membrane Damage: The lysosome is surrounded by a membrane that keeps the digestive enzymes separate from the rest of the cell. If this membrane is damaged, the enzymes could leak out and damage the cell, or the undigested material could escape into the cell. This could be caused by physical damage, exposure to toxins, or infection.

3. pH Imbalance: The inside of a lysosome is acidic, which is necessary for the enzymes to function. If the pH balance is off, the enzymes may not work properly. This could be due to a problem with the cell's proton pumps, which are responsible for maintaining the acidic environment.

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Key Concepts

Here are the essential concepts you must grasp in order to answer the question correctly.

Lysosomal Function

Lysosomes are membrane-bound organelles that contain digestive enzymes responsible for breaking down waste materials and cellular debris. They play a crucial role in cellular homeostasis by recycling macromolecules and removing damaged organelles. Proper lysosomal function is essential for maintaining cellular health and preventing the accumulation of undigested materials.

Lysosomal Storage Diseases

Lysosomal storage diseases are a group of inherited metabolic disorders caused by enzyme deficiencies within lysosomes. These deficiencies lead to the accumulation of undigested substrates, resulting in cellular dysfunction and various clinical symptoms. Examples include Tay-Sachs disease and Gaucher's disease, which highlight the importance of specific enzymes for lysosomal activity.

Autophagy

Autophagy is a cellular process that involves the degradation and recycling of cellular components through lysosomes. It is essential for maintaining cellular homeostasis, especially during stress conditions. Defects in autophagy can lead to the accumulation of damaged organelles and proteins, contributing to lysosomal dysfunction and the buildup of undigested materials.

Related Practice

Textbook Question

Cells that line your intestines are known to possess a large number of membrane proteins that transport small molecules and ions across the plasma membrane. Which of the following cell structures would you expect to be required for this characteristic of the cells?

a. The endoplasmic reticulum

b. Peroxisomes

c. Lysosomes

d. The cell wall

Textbook Question

Most of the proteins that enter the nucleus possess a nuclear localization signal (NLS), even if they are small enough to pass through the nuclear pore complex unhindered. Why would a small protein have an NLS, when it naturally diffuses across the nuclear pore complex without one?

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Textbook Question

The eukaryotic cytoskeleton is a highly dynamic network of filaments and motor proteins. Which of the following correctly describe activities of these cytoskeletal components? Select True or False for each statement.

T/FMyosin motors walk toward the plus ends of intermediate filaments.

T/FDynein motors are responsible for the whip-like movement of eukaryotic flagella.

T/FKinesin motors move vesicles along tracks toward the microtubule-organizing center.

T/FActin filaments are required for cytoplasmic streaming.

Textbook Question

George Palade's research group used the pulse–chase assay to elucidate the secretory pathway in pancreatic cells. If they had instead performed this assay on muscle cells, where would you expect the labeled proteins to end up during the chase?

(Muscle cells consist primarily of actin and myosin filaments and have high energy demands for muscle contraction.)