NameElongation of very long chain fatty acids protein 4
Synonyms
  • 2.3.1.199
  • 3-keto acyl-CoA synthase ELOVL4
  • ELOVL FA elongase 4
  • ELOVL fatty acid elongase 4
  • Very long chain 3-ketoacyl-CoA synthase 4
  • Very long chain 3-oxoacyl-CoA synthase 4
Gene NameELOVL4
OrganismHuman
Amino acid sequence
>lcl|BSEQ0019253|Elongation of very long chain fatty acids protein 4
MGLLDSEPGSVLNVVSTALNDTVEFYRWTWSIADKRVENWPLMQSPWPTLSISTLYLLFV
WLGPKWMKDREPFQMRLVLIIYNFGMVLLNLFIFRELFMGSYNAGYSYICQSVDYSNNVH
EVRIAAALWWYFVSKGVEYLDTVFFILRKKNNQVSFLHVYHHCTMFTLWWIGIKWVAGGQ
AFFGAQLNSFIHVIMYSYYGLTAFGPWIQKYLWWKRYLTMLQLIQFHVTIGHTALSLYTD
CPFPKWMHWALIAYAISFIFLFLNFYIRTYKEPKKPKAGKTAMNGISANGVSKSEKQLMI
ENGKKQKNGKAKGD
Number of residues314
Molecular Weight36828.905
Theoretical pI9.86
GO Classification
Functions
  • transferase activity
  • G-protein coupled photoreceptor activity
Processes
  • fatty acid elongation, saturated fatty acid
  • very long-chain fatty acid biosynthetic process
  • small molecule metabolic process
  • cellular lipid metabolic process
  • fatty acid biosynthetic process
  • triglyceride biosynthetic process
  • long-chain fatty-acyl-CoA biosynthetic process
  • detection of visible light
Components
  • endoplasmic reticulum
  • integral component of endoplasmic reticulum membrane
General FunctionTransferase activity
Specific FunctionCatalyzes the first and rate-limiting reaction of the four that constitute the long-chain fatty acids elongation cycle. This endoplasmic reticulum-bound enzymatic process, allows the addition of 2 carbons to the chain of long- and very long-chain fatty acids/VLCFAs per cycle. Condensing enzyme that specifically elongates C24:0 and C26:0 acyl-CoAs. May participate to the production of saturated and monounsaturated VLCFAs of different chain lengths that are involved in multiple biological processes as precursors of membrane lipids and lipid mediators. May play a critical role in early brain and skin development.
Pfam Domain Function
Transmembrane Regions42-62 78-98 127-147 165-185 188-208 217-237 247-267
GenBank Protein ID12044051
UniProtKB IDQ9GZR5
UniProtKB Entry NameELOV4_HUMAN
Cellular LocationEndoplasmic reticulum membrane
Gene sequence
>lcl|BSEQ0019254|Elongation of very long chain fatty acids protein 4 (ELOVL4)
ATGGGGCTCCTGGACTCGGAGCCGGGTAGTGTCCTAAACGTAGTGTCCACGGCACTCAAC
GACACGGTAGAGTTCTACCGCTGGACCTGGTCCATCGCAGATAAGCGTGTGGAAAATTGG
CCTCTGATGCAGTCTCCTTGGCCTACACTAAGTATAAGCACTCTTTATCTCCTGTTTGTG
TGGCTGGGTCCAAAATGGATGAAGGACCGAGAACCTTTTCAGATGCGTCTAGTGCTCATT
ATCTATAATTTTGGGATGGTTTTGCTTAACCTCTTTATCTTCAGAGAGTTATTCATGGGA
TCATATAATGCGGGATATAGCTATATTTGCCAGAGTGTGGATTATTCTAATAATGTTCAT
GAAGTCAGGATAGCTGCTGCTCTGTGGTGGTACTTTGTATCTAAAGGAGTTGAGTATTTG
GACACAGTGTTTTTTATTCTGAGAAAGAAAAACAACCAAGTTTCTTTCCTTCATGTGTAT
CATCACTGTACGATGTTTACCTTGTGGTGGATTGGAATTAAGTGGGTTGCAGGAGGACAA
GCATTTTTTGGAGCCCAGTTGAATTCCTTTATCCATGTGATTATGTACTCATACTATGGG
TTAACTGCATTTGGCCCATGGATTCAGAAATATCTTTGGTGGAAACGATACCTGACTATG
TTGCAACTGATTCAATTCCATGTGACCATTGGGCACACGGCACTGTCTCTTTACACTGAC
TGCCCCTTCCCCAAATGGATGCACTGGGCTCTAATTGCCTATGCAATCAGCTTCATATTT
CTCTTTCTTAACTTCTACATTCGGACATACAAAGAGCCTAAGAAACCAAAAGCTGGAAAA
ACAGCCATGAATGGTATTTCAGCAAATGGTGTGAGCAAATCAGAAAAACAACTCATGATA
GAAAATGGAAAAAAGCAGAAAAATGGAAAAGCAAAAGGAGATTAA
GenBank Gene IDAF279654
GeneCard IDNot Available
GenAtlas IDELOVL4
HGNC IDHGNC:14415
Chromosome Location6
Locus6q14
References
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  2. 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. 11581213
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