Acyl-coa oxidase

Definition

Acyl-CoA oxidase is an enzyme located in peroxisomes that plays a vital role in the metabolism of fatty acids by catalyzing the first step in the β-oxidation pathway of very long-chain fatty acids. This enzyme facilitates the conversion of acyl-CoA into trans-2-enoyl-CoA while simultaneously producing hydrogen peroxide as a byproduct. The activity of acyl-CoA oxidase is essential for the proper breakdown of fatty acids, which is crucial for energy production and maintaining cellular functions.

5 Must Know Facts For Your Next Test

1. Acyl-CoA oxidase is the first enzyme in the β-oxidation pathway specifically for very long-chain fatty acids, indicating its importance in fatty acid metabolism.
2. The hydrogen peroxide generated during the reaction catalyzed by acyl-CoA oxidase is later decomposed by other enzymes, like catalase, to prevent cellular damage.
3. Deficiencies or mutations in the acyl-CoA oxidase gene can lead to metabolic disorders, such as peroxisomal biogenesis disorders or Zellweger syndrome.
4. Acyl-CoA oxidase is specific for fatty acids of varying chain lengths; however, it primarily acts on those with longer chains.
5. The activity of acyl-CoA oxidase can be influenced by various factors, including the availability of substrates and cellular energy status.

Review Questions

• How does acyl-CoA oxidase contribute to fatty acid metabolism within peroxisomes?
  • Acyl-CoA oxidase initiates the β-oxidation pathway for very long-chain fatty acids within peroxisomes by converting acyl-CoA into trans-2-enoyl-CoA. This reaction not only allows for the breakdown of these fatty acids but also generates hydrogen peroxide as a byproduct. The function of this enzyme is critical in regulating energy production and lipid metabolism in cells.
• Discuss the implications of acyl-CoA oxidase deficiencies on human health and metabolism.
  • Deficiencies in acyl-CoA oxidase can lead to severe metabolic disorders, such as Zellweger syndrome and other peroxisomal biogenesis disorders. These conditions are characterized by an accumulation of very long-chain fatty acids due to impaired fatty acid metabolism, resulting in various health issues including neurological impairment and organ dysfunction. Understanding these implications highlights the importance of this enzyme in overall metabolic health.
• Evaluate the potential impact of manipulating acyl-CoA oxidase activity on metabolic diseases associated with fatty acid oxidation.
  • Manipulating the activity of acyl-CoA oxidase could provide therapeutic avenues for treating metabolic diseases linked to impaired fatty acid oxidation. For instance, enhancing its activity might improve the breakdown of very long-chain fatty acids, potentially alleviating symptoms in patients with related disorders. Conversely, inhibiting its action could have applications in conditions where reduced fatty acid metabolism is desired. Careful evaluation of such interventions would be necessary to ensure safety and efficacy in managing these metabolic challenges.

Related terms

Peroxisome: A small, membrane-bound organelle in cells responsible for breaking down fatty acids and detoxifying harmful substances.

β-oxidation: A metabolic process that breaks down fatty acids into acetyl-CoA units, providing energy for the cell.

Hydrogen peroxide: A reactive oxygen species produced as a byproduct of various metabolic reactions, including those involving acyl-CoA oxidase.