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Background
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ACOX1 is the first enzyme of the fatty acid beta-oxidation pathway, which catalyzes the desaturation of acyl-CoAs to 2-trans-enoyl-CoAs. It donates
electrons directly to molecular oxygen, thereby producing hydrogen peroxide. Defects in this gene result in pseudoneonatal adrenoleukodystrophy, a disease that is characterized by accumulation of very long chain fatty acids.
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Background
References
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- Strausberg, R.L., et al., Proc. Natl. Acad. Sci. U.S.A. 99(26):16899-16903 (2002).
- Chu, R., et al., J. Biol. Chem. 270(9):4908-4915 (1995).
- Aoyama, T., et al., Biochem. Biophys. Res. Commun. 198(3):1113-1118 (1994).
- Fournier, B., et al., J. Clin. Invest. 94(2):526-531 (1994).
- Varanasi, U., et al., Proc. Natl. Acad. Sci. U.S.A. 91(8):3107-3111 (1994).
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