MCAT® ExamBiological and Biochemical Foundations of Living SystemsMedium

A patient is diagnosed with a rare genetic disorder where their cells are unable to properly degrade long-chain fatty acids, leading to their accumulation and cellular dysfunction. Analysis reveals a defect in an organelle responsible for detoxification and lipid metabolism. Which organelle is most likely affected in this patient?

  1. ARough Endoplasmic Reticulum
  2. BMitochondria
  3. CLysosomes
  4. DPeroxisomes
Show answer & explanation

Correct answer: D. Peroxisomes

Peroxisomes are responsible for the beta-oxidation of very long-chain fatty acids, detoxification of harmful substances, and synthesis of certain lipids. A defect in peroxisomes would lead to the accumulation of long-chain fatty acids.

Why the other options are wrong

  • A. Rough Endoplasmic Reticulum is involved in protein synthesis and modification, not fatty acid degradation.
  • B. Mitochondria are involved in cellular respiration and ATP production, and also beta-oxidation of shorter fatty acids, but peroxisomes handle very long-chain fatty acids.
  • C. Lysosomes are primarily involved in the degradation of waste materials and cellular debris, not specifically long-chain fatty acid breakdown.

Peroxisome Function

Membrane-bound organelles involved in metabolic processes, including the breakdown of fatty acids (especially very long-chain fatty acids) and detoxification of harmful substances.

  • Contain oxidative enzymes (e.g., catalase, oxidases).
  • Perform beta-oxidation of very long-chain fatty acids.
  • Detoxify hydrogen peroxide and other harmful compounds.
  • Involved in lipid biosynthesis.

Memory trick: Peroxisomes are the peroxi-cleaners for fatty acids.

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