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Identification
HMDB Protein ID HMDBP04479
Secondary Accession Numbers
  • 10072
Name Elongation of very long chain fatty acids protein 4
Synonyms
  1. 3-keto acyl-CoA synthase ELOVL4
  2. ELOVL fatty acid elongase 4
  3. Very-long-chain 3-oxoacyl-CoA synthase 4
  4. ELOVL FA elongase 4
Gene Name ELOVL4
Protein Type Enzyme
Biological Properties
General Function Involved in G-protein coupled photoreceptor activity
Specific Function Condensing enzyme that elongates saturated and monounsaturated very long chain fatty acids (VLCFAs). Elongates C24:0 and C26:0 acyl-CoAs. Seems to represent a photoreceptor-specific component of the fatty acid elongation system residing on the endoplasmic reticulum. May be implicated in docosahexaenoic acid (DHA) biosynthesis, which requires dietary consumption of the essential alpha-linolenic acid and a subsequent series of three elongation steps. May play a critical role in early brain and skin development.
Pathways
  • Alpha Linolenic Acid and Linoleic Acid Metabolism
  • Fatty acid elongation
Reactions
A very-long-chain acyl-CoA + Malonyl-CoA → Coenzyme A + a very-long-chain 3-oxoacyl-CoA + CO(2) details
Malonyl-CoA + Acyl-CoA → 3-Oxoacyl-CoA + Coenzyme A + Carbon dioxide details
GO Classification
Biological Process
fatty acid elongation, saturated fatty acid
very long-chain fatty acid biosynthetic process
long-chain fatty-acyl-CoA biosynthetic process
triglyceride biosynthetic process
Cellular Component
integral to endoplasmic reticulum membrane
Component
cell part
membrane part
intrinsic to membrane
integral to membrane
Molecular Function
G-protein coupled photoreceptor activity
transferase activity
Cellular Location
  1. Multi-pass membrane protein (Potential)
  2. Endoplasmic reticulum membrane
Gene Properties
Chromosome Location 6
Locus 6q14
SNPs ELOVL4
Gene Sequence
>945 bp
ATGGGGCTCCTGGACTCGGAGCCGGGTAGTGTCCTAAACGTAGTGTCCACGGCACTCAAC
GACACGGTAGAGTTCTACCGCTGGACCTGGTCCATCGCAGATAAGCGTGTGGAAAATTGG
CCTCTGATGCAGTCTCCTTGGCCTACACTAAGTATAAGCACTCTTTATCTCCTGTTTGTG
TGGCTGGGTCCAAAATGGATGAAGGACCGAGAACCTTTTCAGATGCGTCTAGTGCTCATT
ATCTATAATTTTGGGATGGTTTTGCTTAACCTCTTTATCTTCAGAGAGTTATTCATGGGA
TCATATAATGCGGGATATAGCTATATTTGCCAGAGTGTGGATTATTCTAATAATGTTCAT
GAAGTCAGGATAGCTGCTGCTCTGTGGTGGTACTTTGTATCTAAAGGAGTTGAGTATTTG
GACACAGTGTTTTTTATTCTGAGAAAGAAAAACAACCAAGTTTCTTTCCTTCATGTGTAT
CATCACTGTACGATGTTTACCTTGTGGTGGATTGGAATTAAGTGGGTTGCAGGAGGACAA
GCATTTTTTGGAGCCCAGTTGAATTCCTTTATCCATGTGATTATGTACTCATACTATGGG
TTAACTGCATTTGGCCCATGGATTCAGAAATATCTTTGGTGGAAACGATACCTGACTATG
TTGCAACTGATTCAATTCCATGTGACCATTGGGCACACGGCACTGTCTCTTTACACTGAC
TGCCCCTTCCCCAAATGGATGCACTGGGCTCTAATTGCCTATGCAATCAGCTTCATATTT
CTCTTTCTTAACTTCTACATTCGGACATACAAAGAGCCTAAGAAACCAAAAGCTGGAAAA
ACAGCCATGAATGGTATTTCAGCAAATGGTGTGAGCAAATCAGAAAAACAACTCATGATA
GAAAATGGAAAAAAGCAGAAAAATGGAAAAGCAAAAGGAGATTAA
Protein Properties
Number of Residues 314
Molecular Weight 36828.905
Theoretical pI 9.468
Pfam Domain Function
Signals Not Available
Transmembrane Regions Not Available
Protein Sequence
>Elongation of very long chain fatty acids protein 4
MGLLDSEPGSVLNVVSTALNDTVEFYRWTWSIADKRVENWPLMQSPWPTLSISTLYLLFV
WLGPKWMKDREPFQMRLVLIIYNFGMVLLNLFIFRELFMGSYNAGYSYICQSVDYSNNVH
EVRIAAALWWYFVSKGVEYLDTVFFILRKKNNQVSFLHVYHHCTMFTLWWIGIKWVAGGQ
AFFGAQLNSFIHVIMYSYYGLTAFGPWIQKYLWWKRYLTMLQLIQFHVTIGHTALSLYTD
CPFPKWMHWALIAYAISFIFLFLNFYIRTYKEPKKPKAGKTAMNGISANGVSKSEKQLMI
ENGKKQKNGKAKGD
GenBank ID Protein Not Available
UniProtKB/Swiss-Prot ID Q9GZR5
UniProtKB/Swiss-Prot Entry Name ELOV4_HUMAN
PDB IDs Not Available
GenBank Gene ID AF277094
GeneCard ID ELOVL4
GenAtlas ID ELOVL4
HGNC ID HGNC:14415
References
General References
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  3. Gerhard DS, Wagner L, Feingold EA, Shenmen CM, Grouse LH, Schuler G, Klein SL, Old S, Rasooly R, Good P, Guyer M, Peck AM, Derge JG, Lipman D, Collins FS, Jang W, Sherry S, Feolo M, Misquitta L, Lee E, Rotmistrovsky K, Greenhut SF, Schaefer CF, Buetow K, Bonner TI, Haussler D, Kent J, Kiekhaus M, Furey T, Brent M, Prange C, Schreiber K, Shapiro N, Bhat NK, Hopkins RF, Hsie F, Driscoll T, Soares MB, Casavant TL, Scheetz TE, Brown-stein MJ, Usdin TB, Toshiyuki S, Carninci P, Piao Y, Dudekula DB, Ko MS, Kawakami K, Suzuki Y, Sugano S, Gruber CE, Smith MR, Simmons B, Moore T, Waterman R, Johnson SL, Ruan Y, Wei CL, Mathavan S, Gunaratne PH, Wu J, Garcia AM, Hulyk SW, Fuh E, Yuan Y, Sneed A, Kowis C, Hodgson A, Muzny DM, McPherson J, Gibbs RA, Fahey J, Helton E, Ketteman M, Madan A, Rodrigues S, Sanchez A, Whiting M, Madari A, Young AC, Wetherby KD, Granite SJ, Kwong PN, Brinkley CP, Pearson RL, Bouffard GG, Blakesly RW, Green ED, Dickson MC, Rodriguez AC, Grimwood J, Schmutz J, Myers RM, Butterfield YS, Griffith M, Griffith OL, Krzywinski MI, Liao N, Morin R, Palmquist D, Petrescu AS, Skalska U, Smailus DE, Stott JM, Schnerch A, Schein JE, Jones SJ, Holt RA, Baross A, Marra MA, Clifton S, Makowski KA, Bosak S, Malek J: The status, quality, and expansion of the NIH full-length cDNA project: the Mammalian Gene Collection (MGC). Genome Res. 2004 Oct;14(10B):2121-7. [PubMed:15489334 ]
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  5. Edwards AO, Donoso LA, Ritter R 3rd: A novel gene for autosomal dominant Stargardt-like macular dystrophy with homology to the SUR4 protein family. Invest Ophthalmol Vis Sci. 2001 Oct;42(11):2652-63. [PubMed:11581213 ]
  6. Rivolta C, Ayyagari R, Sieving PA, Berson EL, Dryja TP: Evaluation of the ELOVL4 gene in patients with autosomal recessive retinitis pigmentosa and Leber congenital amaurosis. Mol Vis. 2003 Feb 18;9:49-51. [PubMed:12592226 ]