| Entry |
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| Name |
Fatty acid elongation
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| Class |
Metabolism; Lipid metabolism
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| Pathway map |
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| Module |
| Fatty acid biosynthesis, elongation, mitochondria [PATH: ko00062] | | Fatty acid biosynthesis, elongation, endoplasmic reticulum [PATH: ko00062] |
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| Disease |
| Neuronal ceroid lipofuscinosis | | Hyperargininemia | | Progressive myoclonic epilepsy (PME) | | Stargardt disease (STGD) |
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| Orthology |
| acetyl-CoA acyltransferase 2 [EC: 2.3.1.16] | | acetyl-CoA acyltransferase [EC: 2.3.1.16] | | 3-hydroxyacyl-CoA dehydrogenase [EC: 1.1.1.35] | | enoyl-CoA hydratase / long-chain 3-hydroxyacyl-CoA dehydrogenase [EC: 1.1.1.211 4.2.1.17] | | | | mitochondrial trans-2-enoyl-CoA reductase [EC: 1.3.1.38] | | palmitoyl-protein thioesterase [EC: 3.1.2.22] | | elongation of very long chain fatty acids protein 1 [EC: 2.3.1.199] | | elongation of very long chain fatty acids protein 2 [EC: 2.3.1.199] | | elongation of very long chain fatty acids protein 3 [EC: 2.3.1.199] | | elongation of very long chain fatty acids protein 4 [EC: 2.3.1.199] | | elongation of very long chain fatty acids protein 5 [EC: 2.3.1.199] | | elongation of very long chain fatty acids protein 6 [EC: 2.3.1.199] | | elongation of very long chain fatty acids protein 7 [EC: 2.3.1.199] | | | | | | | | 17beta-estradiol 17-dehydrogenase / very-long-chain 3-oxoacyl-CoA reductase [EC: 1.1.1.330 1.1.1.62] | | very-long-chain (3R)-3-hydroxyacyl-[acyl-carrier protein] dehydratase [EC: 4.2.1.134] | | very-long-chain enoyl-CoA reductase [EC: 1.3.1.93] | | palmitoyl-CoA hydrolase [EC: 3.1.2.2] |
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| Compound |
| Acetyl-CoA | | Acyl-CoA | | Malonyl-CoA | | Butanoyl-CoA | | Palmitoyl-CoA | | Fatty acid | | Hexadecanoic acid | | 3-Oxoacyl-CoA | | 2,3-Dehydroacyl-CoA | | Long-chain fatty acid | | (3S)-3-Hydroxyacyl-CoA | | trans-2,3-Dehydroacyl-CoA | | Lauroyl-CoA | | Octanoyl-CoA | | Tetradecanoyl-CoA | | Long-chain acyl-CoA | | 2-trans-Dodecenoyl-CoA | | (S)-3-Hydroxyhexadecanoyl-CoA | | 3-Oxopalmitoyl-CoA | | (S)-3-Hydroxytetradecanoyl-CoA | | 3-Oxotetradecanoyl-CoA | | (S)-3-Hydroxydodecanoyl-CoA | | 3-Oxododecanoyl-CoA | | (S)-Hydroxydecanoyl-CoA | | 3-Oxodecanoyl-CoA | | (S)-3-Hydroxyoctanoyl-CoA | | 3-Oxooctanoyl-CoA | | (S)-Hydroxyhexanoyl-CoA | | 3-Oxohexanoyl-CoA | | Hexanoyl-CoA | | trans-Hex-2-enoyl-CoA | | trans-Hexadec-2-enoyl-CoA | | trans-Tetradec-2-enoyl-CoA | | Decanoyl-CoA | | trans-Dec-2-enoyl-CoA | | trans-Oct-2-enoyl-CoA |
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| Reference |
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| Authors |
Seubert W, Lamberts I, Kramer R, Ohly B |
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| Title |
On the mechanism of malonyl-CoA-independent fatty acid synthesis. I. The mechanism of elongation of long-chain fatty acids by acetyl-CoA. |
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| Journal |
Biochim Biophys Acta 164:498-517 (1968) |
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| Reference |
|
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| Authors |
El-Fakhri M, Middleton B |
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| Title |
The existence of an inner-membrane-bound, long acyl-chain-specific 3-hydroxyacyl-CoA dehydrogenase in mammalian mitochondria. |
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| Journal |
Biochim Biophys Acta 713:270-9 (1982) |
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| Reference |
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| Authors |
Cassagne C, Lessire R, Bessoule JJ, Moreau P, Creach A, Schneider F, Sturbois B |
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| Title |
Biosynthesis of very long chain fatty acids in higher plants. |
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| Journal |
Prog Lipid Res 33:55-69 (1994) |
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| Reference |
|
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| Authors |
Jakobsson A, Westerberg R, Jacobsson A. |
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| Title |
Fatty acid elongases in mammals: their regulation and roles in metabolism. |
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| Journal |
Prog Lipid Res 45:237-49 (2006) |
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| Reference |
|
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| Authors |
Blacklock BJ, Jaworski JG |
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| Title |
Substrate specificity of Arabidopsis 3-ketoacyl-CoA synthases. |
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| Journal |
Biochem Biophys Res Commun 346:583-90 (2006) |
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| Reference |
|
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| Authors |
Shepherd T, Wynne Griffiths D |
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| Title |
The effects of stress on plant cuticular waxes. |
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| Journal |
New Phytol 171:469-99 (2006) |
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| Reference |
|
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| Authors |
Riezman H |
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| Title |
The long and short of fatty acid synthesis. |
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| Journal |
Cell 130:587-8 (2007) |
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| Reference |
|
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| Authors |
Raffaele S, Leger A, Roby D |
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| Title |
Very long chain fatty acid and lipid signaling in the response of plants to pathogens. |
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| Journal |
Plant Signal Behav 4:94-9 (2009) |
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