ELOVL1,4 elongate TCS-CoA and Mal-CoA to 3OHC-CoA

Stable Identifier
R-HSA-548830
Type
Reaction [transition]
Species
Homo sapiens
Compartment
cytosol, endoplasmic reticulum membrane
Synonyms
H(+) + malonyl-CoA + tetracosanoyl-CoA => 3-oxohexacosanoyl-CoA + CO2 + CoA, lignoceroyl-CoA + malonyl-CoA => 3-oxocerotoyl-CoA + CO2 + CoASH
ReviewStatus
5/5
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ELOVL1,4 elongate TCS-CoA and Mal-CoA to 3OHC-CoA
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Elongation of very long chain fatty acids proteins 1, 4 (ELOVL1,4) catalyse the elongation of lignoceroyl-CoA (TCS-CoA) and malonyl-CoA (Mal-CoA) to form 3-oxocerotoyl-CoA (3OHC-CoA). ELOVL4 is abundant in retinal cells, where it is localized to the endoplasmic reticulum membrane (Grayson & Molday 2005). The catalytic activity of ELOVL4 has not been examined directly but is inferred from that of the homologous mouse protein, which is also active on polyunsaturated fatty acids (PUFAs) (PUFAs) (Agbada et al. 2008).
Literature References
PubMed ID Title Journal Year
16036915 Dominant negative mechanism underlies autosomal dominant Stargardt-like macular dystrophy linked to mutations in ELOVL4

Grayson, C, Molday, RS

J Biol Chem 2005
18728184 Role of Stargardt-3 macular dystrophy protein (ELOVL4) in the biosynthesis of very long chain fatty acids

Agbaga, MP, Brush, RS, Mandal, MN, Henry, K, Elliott, MH, Anderson, RE

Proc Natl Acad Sci U S A 2008
Participants
Input
Output
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as an event of
Catalyst Activity

fatty acid elongase activity of ELOVL1,4 [endoplasmic reticulum membrane]

Physical Entity
ELOVL1,4 [endoplasmic reticulum membrane] (Homo sapiens)
Activity
fatty acid elongase activity (GO:0009922)
Inferred From
lignoceroyl-CoA + malonyl-CoA => 3-oxocerotoyl-CoA + CO2 + CoASH [Elovl4] (Mus musculus)
Cross References
RHEA
Authored
D'Eustachio, P  (2010-03-15)
Reviewed
D'Eustachio, P  (2015-02-22)
Hill, DP  (2024-04-12)
Created
D'Eustachio, P  (2010-03-15)
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