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Questions and Answers
What does fatty change (steatosis) primarily indicate about triglyceride accumulation?
What does fatty change (steatosis) primarily indicate about triglyceride accumulation?
Which factor is NOT commonly associated with causing fatty change in cells?
Which factor is NOT commonly associated with causing fatty change in cells?
In which organ is fatty change most frequently observed?
In which organ is fatty change most frequently observed?
Which of the following organs is least likely to display fatty change?
Which of the following organs is least likely to display fatty change?
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Which description best fits the cause of steatosis?
Which description best fits the cause of steatosis?
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What is the primary cause of accumulation of abnormal endogenous substances related to α1-antitrypsin?
What is the primary cause of accumulation of abnormal endogenous substances related to α1-antitrypsin?
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Which of the following statements correctly identifies a mechanism leading to the abnormality of α1-antitrypsin?
Which of the following statements correctly identifies a mechanism leading to the abnormality of α1-antitrypsin?
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What outcome can result from defective folding or packaging of proteins like α1-antitrypsin?
What outcome can result from defective folding or packaging of proteins like α1-antitrypsin?
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Which factor is NOT typically involved in the accumulation of abnormal endogenous substances?
Which factor is NOT typically involved in the accumulation of abnormal endogenous substances?
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How does a mutation in α1-antitrypsin contribute to its abnormal accumulation?
How does a mutation in α1-antitrypsin contribute to its abnormal accumulation?
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Study Notes
Intracellular Accumulation and Calcification
- Cells accumulate abnormal substances under some circumstances, which can be harmless or cause injury.
- Accumulated substances can be found in the cytoplasm, organelles (typically lysosomes), or the nucleus.
Mechanisms
- Inadequate removal of a normal substance: Defects in packaging/transport mechanisms, like fatty liver change, can lead to accumulation.
- Abnormal endogenous substance accumulation: Genetic or acquired defects in folding, packaging, transport, or secretion of substances (e.g., mutated a1-antitrypsin).
- Failure to degrade a metabolite: Inherited enzyme deficiencies (e.g., lysosomal storage diseases, glycogen storage diseases) lead to buildup of metabolites.
- Abnormal exogenous substance accumulation: Cells can't degrade or transport substances (e.g., carbon or silica particles).
Fatty Change (Steatosis)
- Abnormal accumulation of triglycerides in parenchymal cells.
- Caused by excessive intake or defects in synthesis/transport of proteins.
- Commonly occurs in the liver, but can also affect heart, skeletal muscle, and other organs.
- Alcohol abuse and diabetes are frequent causes.
Cholesterol and Cholesteryl Esters
- Cholesterol deposits occur due to defective catabolism or excessive intake.
- Seen in macrophages and smooth muscle cells of vessel walls (atherosclerosis).
Proteins
- Trace amounts of albumin normally filtered and reabsorbed in the kidneys.
- Heavy protein leakage (e.g., nephrotic syndrome) leads to increased reabsorption, forming pink/hyaline cytoplasmic droplets.
- Accumulation of immunoglobulins (Russell bodies).
- Neurofibrillary tangles are aggregates of microtubules binding protein tau.
Pigments (Colored Substances)
- Exogenous: Anthracosis (carbon accumulation) is common.
- Endogenous:
- Lipofuscin ("wear-and-tear pigment"): insoluble brownish-yellow material accumulating with age or atrophy.
- Melanin: endogenous brown-black pigment.
- Hemosiderin: hemoglobin-derived, golden/brown granular pigment; arises from excess iron.
Pathologic Calcification
- Dystrophic: Abnormal calcium deposition in dead/dying tissues; often asymptomatic. Can cause dysfunction in affected organs.
- Metastatic: Calcium deposition in healthy tissues due to hypercalcemia. Frequently affects vessels, kidneys, lungs, and gastric mucosa.
Cellular Aging
- Cellular aging is a decline in lifespan and functional capacity.
- Mechanisms include DNA damage and reduced cellular replication leading to replicative senescence (cellular arrest due to shortened telomeres).
- Defective protein homeostasis contributes via increased protein misfolding.
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Description
This quiz covers key concepts related to fatty change (steatosis) and the implications of α1-antitrypsin accumulation in cells. Questions explore the causes, mechanisms, and outcomes of these conditions, aimed at enhancing understanding of liver pathology and protein misfolding disorders.