KEGG   PATHWAY: hsa00062Help
Entry
hsa00062                    Pathway                                

Name
Fatty acid elongation - Homo sapiens (human)
Class
Metabolism; Lipid metabolism
BRITE hierarchy
Pathway map
Fatty acid elongation
hsa00062

All organismsOrtholog table
Module
Fatty acid biosynthesis, elongation, mitochondria [PATH:hsa00062]
Fatty acid biosynthesis, elongation, endoplasmic reticulum [PATH:hsa00062]
Disease
H00149  
Neuronal ceroid lipofuscinosis
H00186  
Hyperargininemia
H00186  
Hyperargininemia
H00810  
Progressive myoclonic epilepsy (PME/ EPM)
H00819  
Stargardt disease (STGD)
H01364  
3-Hydroxyacyl-CoA dehydrogenase deficiency
Organism
Homo sapiens (human) [GN:hsa]
Gene
10449  
ACAA2; acetyl-CoA acyltransferase 2 [KO:K07508] [EC:2.3.1.16]
3032  
HADHB; hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), beta subunit [KO:K07509] [EC:2.3.1.16]
3033  
HADH; hydroxyacyl-CoA dehydrogenase [KO:K00022] [EC:1.1.1.35]
3030  
HADHA; hydroxyacyl-CoA dehydrogenase/3-ketoacyl-CoA thiolase/enoyl-CoA hydratase (trifunctional protein), alpha subunit [KO:K07515] [EC:1.1.1.211 4.2.1.17]
1892  
ECHS1; enoyl-CoA hydratase, short chain, 1, mitochondrial [KO:K07511] [EC:4.2.1.17]
51102  
MECR; mitochondrial trans-2-enoyl-CoA reductase [KO:K07512] [EC:1.3.1.38]
5538  
PPT1; palmitoyl-protein thioesterase 1 [KO:K01074] [EC:3.1.2.22]
9374  
PPT2; palmitoyl-protein thioesterase 2 [KO:K01074] [EC:3.1.2.22]
64834  
ELOVL1; ELOVL fatty acid elongase 1 [KO:K10247] [EC:2.3.1.199]
54898  
ELOVL2; ELOVL fatty acid elongase 2 [KO:K10205] [EC:2.3.1.199]
83401  
ELOVL3; ELOVL fatty acid elongase 3 [KO:K10248] [EC:2.3.1.199]
6785  
ELOVL4; ELOVL fatty acid elongase 4 [KO:K10249] [EC:2.3.1.199]
60481  
ELOVL5; ELOVL fatty acid elongase 5 [KO:K10244] [EC:2.3.1.199]
79071  
ELOVL6; ELOVL fatty acid elongase 6 [KO:K10203] [EC:2.3.1.199]
79993  
ELOVL7; ELOVL fatty acid elongase 7 [KO:K10250] [EC:2.3.1.199]
51144  
HSD17B12; hydroxysteroid (17-beta) dehydrogenase 12 [KO:K10251] [EC:1.1.1.330 1.1.1.62]
201562  
HACD2; 3-hydroxyacyl-CoA dehydratase 2 [KO:K10703] [EC:4.2.1.134]
9200  
HACD1; 3-hydroxyacyl-CoA dehydratase 1 [KO:K10703] [EC:4.2.1.134]
401494  
HACD4; 3-hydroxyacyl-CoA dehydratase 4 [KO:K10703] [EC:4.2.1.134]
51495  
HACD3; 3-hydroxyacyl-CoA dehydratase 3 [KO:K10703] [EC:4.2.1.134]
9524  
TECR; trans-2,3-enoyl-CoA reductase [KO:K10258] [EC:1.3.1.93]
122970  
ACOT4; acyl-CoA thioesterase 4 [KO:K01068] [EC:3.1.2.2]
10965  
ACOT2; acyl-CoA thioesterase 2 [KO:K01068] [EC:3.1.2.2]
641371  
ACOT1; acyl-CoA thioesterase 1 [KO:K01068] [EC:3.1.2.2]
11332  
ACOT7; acyl-CoA thioesterase 7 [KO:K17360] [EC:3.1.2.2]
Compound
C00024  
Acetyl-CoA
C00040  
Acyl-CoA
C00083  
Malonyl-CoA
C00136  
Butanoyl-CoA
C00154  
Palmitoyl-CoA
C00162  
Fatty acid
C00249  
Hexadecanoic acid
C00264  
3-Oxoacyl-CoA
C00605  
2,3-Dehydroacyl-CoA
C00638  
Long-chain fatty acid
C00640  
(3S)-3-Hydroxyacyl-CoA
C00658  
trans-2,3-Dehydroacyl-CoA
C01832  
Lauroyl-CoA
C01944  
Octanoyl-CoA
C02593  
Tetradecanoyl-CoA
C02843  
Long-chain acyl-CoA
C03221  
2-trans-Dodecenoyl-CoA
C05258  
(S)-3-Hydroxyhexadecanoyl-CoA
C05259  
3-Oxopalmitoyl-CoA
C05260  
(S)-3-Hydroxytetradecanoyl-CoA
C05261  
3-Oxotetradecanoyl-CoA
C05262  
(S)-3-Hydroxydodecanoyl-CoA
C05263  
3-Oxododecanoyl-CoA
C05264  
(S)-Hydroxydecanoyl-CoA
C05265  
3-Oxodecanoyl-CoA
C05266  
(S)-3-Hydroxyoctanoyl-CoA
C05267  
3-Oxooctanoyl-CoA
C05268  
(S)-Hydroxyhexanoyl-CoA
C05269  
3-Oxohexanoyl-CoA
C05270  
Hexanoyl-CoA
C05271  
trans-Hex-2-enoyl-CoA
C05272  
trans-Hexadec-2-enoyl-CoA
C05273  
trans-Tetradec-2-enoyl-CoA
C05274  
Decanoyl-CoA
C05275  
trans-Dec-2-enoyl-CoA
C05276  
trans-Oct-2-enoyl-CoA
C20876  
Very-long-chain acyl-CoA
C20877  
Very-long-chain 3-oxoacyl-CoA
C20878  
Very-long-chain (3R)-3-hydroxyacyl-CoA
C20879  
Very-long-chain trans-2,3-dehydroacyl-CoA
Reference
PMID:4387390
  Authors
Seubert W, Lamberts I, Kramer R, Ohly B
  Title
On the mechanism of malonyl-CoA-independent fatty acid synthesis. I. The mechanism of elongation of long-chain fatty acids by acetyl-CoA.
  Journal
Biochim Biophys Acta 164:498-517 (1968)
Reference
PMID:7150615
  Authors
El-Fakhri M, Middleton B
  Title
The existence of an inner-membrane-bound, long acyl-chain-specific 3-hydroxyacyl-CoA dehydrogenase in mammalian mitochondria.
  Journal
Biochim Biophys Acta 713:270-9 (1982)
Reference
PMID:8190743
  Authors
Cassagne C, Lessire R, Bessoule JJ, Moreau P, Creach A, Schneider F, Sturbois B
  Title
Biosynthesis of very long chain fatty acids in higher plants.
  Journal
Prog Lipid Res 33:55-69 (1994)
Reference
  Authors
Jakobsson A, Westerberg R, Jacobsson A.
  Title
Fatty acid elongases in mammals: their regulation and roles in metabolism.
  Journal
Prog Lipid Res 45:237-49 (2006)
Reference
  Authors
Blacklock BJ, Jaworski JG
  Title
Substrate specificity of Arabidopsis 3-ketoacyl-CoA synthases.
  Journal
Biochem Biophys Res Commun 346:583-90 (2006)
Reference
  Authors
Shepherd T, Wynne Griffiths D
  Title
The effects of stress on plant cuticular waxes.
  Journal
New Phytol 171:469-99 (2006)
Reference
  Authors
Riezman H
  Title
The long and short of fatty acid synthesis.
  Journal
Cell 130:587-8 (2007)
Reference
  Authors
Raffaele S, Leger A, Roby D
  Title
Very long chain fatty acid and lipid signaling in the response of plants to pathogens.
  Journal
Plant Signal Behav 4:94-9 (2009)
KO pathway
 

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