Names & Taxonomy
- Uniprot ID:
- Q9GZR5
- Entry Name:
- ELOV4_HUMAN
- Status:
- reviewed
- Protein Names:
- Elongation of very long chain fatty acids protein 4 (EC 2.3.1.199) (3-keto acyl-CoA synthase ELOVL4) (ELOVL fatty acid elongase 4) (ELOVL FA elongase 4) (Very long chain 3-ketoacyl-CoA synthase 4) (Very long chain 3-oxoacyl-CoA synthase 4)
- Gene Names:
- ELOVL4
- Gene Names Primary:
- ELOVL4
- Organism:
- Homo sapiens (Human)
Structure
- Length:
- 314
- Sequence:
- MGLLDSEPGSVLNVVSTALNDTVEFYRWTWSIADKRVENWPLMQSPWPTLSISTLYLLFVWLGPKWMKDREPFQMRLVLIIYNFGMVLLNLFIFRELFMGSYNAGYSYICQSVDYSNNVHEVRIAAALWWYFVSKGVEYLDTVFFILRKKNNQVSFLHVYHHCTMFTLWWIGIKWVAGGQAFFGAQLNSFIHVIMYSYYGLTAFGPWIQKYLWWKRYLTMLQLIQFHVTIGHTALSLYTDCPFPKWMHWALIAYAISFIFLFLNFYIRTYKEPKKPKAGKTAMNGISANGVSKSEKQLMIENGKKQKNGKAKGD
- Proteomes:
- UP000005640
Subcellular location
- Subcellular Location:
- Endoplasmic reticulum membrane
Function
- Function:
- Catalyzes 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.
- Pathway:
- Lipid metabolism; fatty acid biosynthesis.
- Catalytic Activity:
- A very-long-chain acyl-CoA + malonyl-CoA = CoA + a very-long-chain 3-oxoacyl-CoA + CO(2).
- Cross Reference Drug Bank:
- DB00132
- Gene Ontology Go:
- endoplasmic reticulum
integral component of endoplasmic reticulum membrane
G-protein coupled photoreceptor activity
transferase activity
cellular lipid metabolic process
detection of visible light
fatty acid biosynthetic process
fatty acid elongation, saturated fatty acid
long-chain fatty-acyl-CoA biosynthetic process
small molecule metabolic process
triglyceride biosynthetic process
very long-chain fatty acid biosynthetic process - Gene Ontology Biological Process:
- cellular lipid metabolic process
detection of visible light
fatty acid biosynthetic process
fatty acid elongation, saturated fatty acid
long-chain fatty-acyl-CoA biosynthetic process
small molecule metabolic process
triglyceride biosynthetic process
very long-chain fatty acid biosynthetic process - Gene Ontology Molecular Function:
- G-protein coupled photoreceptor activity
transferase activity - Gene Ontology Cellular Component:
- endoplasmic reticulum
integral component of endoplasmic reticulum membrane - Keywords:
- Complete proteome
Disease mutation
Endoplasmic reticulum
Fatty acid biosynthesis
Fatty acid metabolism
Glycoprotein
Ichthyosis
Lipid biosynthesis
Lipid metabolism
Membrane
Mental retardation
Neurodegeneration
Polymorphism
Reference proteome
Spinocerebellar ataxia
Stargardt disease
Transferase
Transmembrane
Transmembrane helix