Answer:
In Gaucher disease, which is caused by a deficiency of an enzyme necessary for lipid metabolism, the accumulation of fatty material occurs within various organs of the body, including the spleen, liver, kidneys, lungs, brain, and bone marrow. To understand how internal membranes and organelles of cells are involved in Gaucher disease, we can examine the role of these cellular components in lipid metabolism and storage.
1. Endoplasmic Reticulum (ER): The endoplasmic reticulum is an organelle involved in lipid metabolism. It is responsible for the synthesis and modification of lipids, including the breakdown and recycling of lipid molecules. In Gaucher disease, the deficiency of the enzyme leads to impaired lipid metabolism, potentially affecting the functioning of the ER and its ability to properly process and transport lipids.
2. Lysosomes: Lysosomes are membrane-bound organelles involved in intracellular digestion and waste disposal. They contain various enzymes, including those responsible for breaking down complex molecules such as lipids. In Gaucher disease, the accumulation of fatty material occurs within the lysosomes due to the deficiency of the enzyme necessary for lipid metabolism. This leads to the buildup of lipids and the formation of characteristic Gaucher cells.
3. Golgi Apparatus: The Golgi apparatus plays a role in modifying, sorting, and packaging lipids and other molecules for transport within the cell or secretion outside the cell. Disruption in lipid metabolism caused by the enzyme deficiency in Gaucher disease can affect the normal functioning of the Golgi apparatus and its ability to properly process and transport lipids.
4. Mitochondria: Mitochondria are involved in various metabolic processes, including the breakdown of fatty acids for energy production. Impairment in lipid metabolism due to the enzyme deficiency in Gaucher disease can potentially affect the mitochondrial function and its ability to efficiently utilize lipids for energy production.
Overall, the involvement of internal membranes and organelles in Gaucher disease stems from the disruption in lipid metabolism caused by the deficiency of the necessary enzyme. This disruption can impair the synthesis, processing, and transport of lipids within the cell, leading to the accumulation of fatty material in various organs and tissues affected by the disease.
Step-by-step explanation: