The DOI matches its Crossref record; 5 of the 5 references it cites were found in a registry or an index.
Identification of the peroxisomal β-oxidation enzymes involved in the biosynthesis of docosahexaenoic acid
Open accessAbstract
DHA (C22:6n-3) is an important PUFA implicated in a number of (patho)physiological processes. For a long time, the exact mechanism of DHA formation has remained unclear, but now it is known that it involves the production of tetracosahexaenoic acid (C24:6n-3) from dietary linolenic acid (C18:3n-3) via a series of elongation and desaturation reactions, followed by beta-oxidation of C24:6n-3 to C22:6n-3. Although DHA is deficient in patients lacking peroxisomes, the intracellular site of retroconversion of C24:6n-3 has remained controversial. By making use of fibroblasts from patients with defined mitochondrial and peroxisomal fatty acid oxidation defects, we show in this article that peroxisomes, and not mitochondria, are involved in DHA formation by catalyzing the beta-oxidation of C24:6n-3 to C22:6n-3. Additional studies of fibroblasts from patients with X-linked adrenoleukodystrophy, straight-chain acyl-CoA oxidase (SCOX) deficiency, d-bifunctional protein (DBP) deficiency, and rhizomelic chondrodysplasia punctata type 1, and of fibroblasts from l-bifunctional protein and sterol carrier protein X (SCPx) knockout mice, show that the main enzymes involved in beta-oxidation of C24:6n-3 to C22:6n-3 are SCOX, DBP, and both 3-ketoacyl-CoA thiolase and SCPx. These findings are of importance for the treatment of patients with a defect in peroxisomal beta-oxidation.
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Cites
The Mitochondrial Carnitine Palmitoyltransferase System — From Concept to Molecular Analysis
McGarry, J. D., & Brown, N. F. (1997) · European Journal of Biochemistry · doi:10.1111/j.1432-1033.1997.00001.x · Cited by 1,616
Putative X-linked adrenoleukodystrophy gene shares unexpected homology with ABC transporters
Mosser, J., Douar, A.-M., Sarde, C.-O., Kioschis, P., Feil, R., Moser, H., Poustka, A.-M., Mandel, J.-L., & Aubourg, P. (1993) · Nature · doi:10.1038/361726a0 · Cited by 1,202
The gene responsible for adrenoleukodystrophy encodes a peroxisomal membrane protein
Mosser, J., Lutz, Y., Stoeckel, M. E., Sarde, C. O., Kretz, C., Douar, A. M., Lopez, J., Aubourg, P., & Mandel, J. L. (1994) · Human Molecular Genetics · doi:10.1093/hmg/3.2.265 · Cited by 281
Disorders of mitochondrial fatty acyl‐CoA β‐oxidation
Wanders, R. J. A., Vreken, P., den Boer, M. E. J., Wijburg, F. A., Van Gennip, A. H., & IJlst, L. (1999) · Journal of Inherited Metabolic Disease · doi:10.1023/a:1005504223140 · Cited by 257
Self-citationWanders, R. J. A., Vreken, P., Ferdinandusse, S., Jansen, G. A., Waterham, H. R., van Roermund, C. W. T., & Van Grunsven, E. G. (2001) · Biochemical Society Transactions · doi:10.1042/bst0290250 · Cited by 225
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The plasma lipidomic signature of nonalcoholic steatohepatitis
Puri, P., Wiest, M. M., Cheung, O., Mirshahi, F., Sargeant, C., Min, H., Contos, M. J., Sterling, R. K., Fuchs, M., Zhou, H., et al. (2009) · Hepatology · doi:10.1002/hep.23229 · Cited by 655
Bergé, J.-P., & Barnathan, G. (2005) · Advances in Biochemical Engineering/Biotechnology · doi:10.1007/b135782 · Cited by 401
Polyunsaturated fatty acid synthesis: What will they think of next?
Wallis, J. G., Watts, J. L., & Browse, J. (2002) · Trends in Biochemical Sciences · doi:10.1016/s0968-0004(02)02168-0 · Cited by 375
Clinical, biochemical, and mutational spectrum of peroxisomal acyl-coenzyme A oxidase deficiency
Ferdinandusse, S., Denis, S., Hogenhout, E. M., Koster, J., van Roermund, C. W. T., IJlst, L., Moser, A. B., Wanders, R. J. A., & Waterham, H. R. (2007) · Human Mutation · doi:10.1002/humu.20535 · Cited by 156
Biosynthesis of docosahexaenoic acid (DHA, 22:6-4, 7,10,13,16,19): Two distinct pathways
Qiu, X. (2003) · Prostaglandins Leukotrienes and Essential Fatty Acids · doi:10.1016/s0952-3278(02)00268-5 · Cited by 149
Metadata
- Authors
- Ferdinandusse, S., Denis, S., Mooijer, P. A. W., Zhang, Z., Reddy, J. K., Spector, A. A., & Wanders, R. J. A.
- Venue
- Journal of Lipid Research
- Publisher
- Elsevier BV
- Published
- 2001-12
- Volume
- 42
- Issue
- 12
- Pages
- 1987-1995
- ISSN
- 0022-2275 (ISSN-L)
- OpenAlex
- W2119068792
- Cited by
- 208
- References
- 5
- FWCI
- 8.55
- Type
- journal article
- Language
- en
- Open access
- hybrid
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- The paper
- Ferdinandusse, S., Denis, S., Mooijer, P. A. W., Zhang, Z., Reddy, J. K., Spector, A. A., & Wanders, R. J. A. (2001). Identification of the peroxisomal β-oxidation enzymes involved in the biosynthesis of docosahexaenoic acid. Journal of Lipid Research. https://doi.org/10.1016/s0022-2275(20)31527-3
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Bibliographic data: Semantic Scholar, OpenAlex, Crossref.
Contains information from the Semantic Scholar Academic Graph Datasets, made available under the ODC Attribution License (ODC-BY 1.0).
Semantic Scholar’s data is described in: Kinney, R., et al. (2023). The Semantic Scholar Open Data Platform. arXiv:2301.10140.
