Peroxisomal acyl-coenzyme A oxidase 1

Definition:

Involved in the initial and rate-limiting step of peroxisomal beta-oxidation of straight-chain saturated and unsaturated very-long-chain fatty acids. Catalyzes the desaturation of fatty acyl-CoAs such as palmitoyl-CoA (hexadecanoyl-CoA) to 2-trans-enoyl-CoAs ((2E)-enoyl-CoAs) such as (2E)-hexadecenoyl-CoA, and donates electrons directly to molecular oxygen (O(2)), thereby producing hydrogen peroxide (H(2)O(2)).; [Isoform 1]: Shows highest activity against medium-chain fatty acyl-CoAs. Shows optimum activity with a chain length of 10 carbons (decanoyl-CoA) in vitro.; [Isoform 2]: Is active against a much broader range of substrates and shows activity towards long-chain fatty acyl-CoAs.

References:

[1]. Atsushi Morita, et al. Novel ACOX1 mutations in two siblings with peroxisomal acyl-CoA oxidase deficiency. Brain Dev. 2021 Mar;43(3):475-481. [Content Brief]

[2]. R Chu, et al. Overexpression and characterization of the human peroxisomal acyl-CoA oxidase in insect cells. J Biol Chem. 1995 Mar 3;270(9):4908-15. [Content Brief]

[3]. Hyung-Lok Chung, et al. Loss- or Gain-of-Function Mutations in ACOX1 Cause Axonal Loss via Different Mechanisms. Neuron. 2020 May 20;106(4):589-606.e6. [Content Brief]

[4]. Sacha Ferdinandusse, et al. Clinical, biochemical, and mutational spectrum of peroxisomal acyl-coenzyme A oxidase deficiency. Hum Mutat. 2007 Sep;28(9):904-12. [Content Brief]

[5]. Sacha Ferdinandusse, et al. Identification of the peroxisomal beta-oxidation enzymes involved in the degradation of long-chain dicarboxylic acids. J Lipid Res. 2004 Jun;45(6):1104-11. [Content Brief]

[6]. David Oaxaca-Castillo, et al. Biochemical characterization of two functional human liver acyl-CoA oxidase isoforms 1a and 1b encoded by a single gene. Biochem Biophys Res Commun. 2007 Aug 24;360(2):314-9. [Content Brief]

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