|Other Names||Elongation of very long chain fatty acids protein 4, 3-keto acyl-CoA synthase ELOVL4, ELOVL fatty acid elongase 4, ELOVL FA elongase 4, Very-long-chain 3-oxoacyl-CoA synthase 4, ELOVL4|
|Format||Synthetic peptide was lyophilized with 100% acetonitrile and is supplied as a powder. Reconstitute with 0.1 ml DI water for a final concentration of 1 mg/ml.|
|Storage||Maintain refrigerated at 2-8°C for up to 6 months. For long term storage store at -20°C.|
|Precautions||This product is for research use only. Not for use in diagnostic or therapeutic procedures.|
|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.|
|Cellular Location||Endoplasmic reticulum membrane; Multi- pass membrane protein|
|Tissue Location||Expressed in the retina and at much lower level in the brain. Ubiquitous, highest expression in thymus, followed by testis, small intestine, ovary, and prostate. Little or no expression in heart, lung, liver, or leukocates|
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ELOVL4 is a membrane-bound protein which is amember of the ELO family, proteins which participate in thebiosynthesis of fatty acids. Consistent with the expression of theencoded protein in photoreceptor cells of the retina, mutations andsmall deletions in this gene are associated with Stargardt-likemacular dystrophy (STGD3) and autosomal dominant Stargardt-likemacular dystrophy (ADMD), also referred to as autosomal dominantatrophic macular degeneration.
Kasperaviciute, D., et al. Brain 133 (PT 7), 2136-2147 (2010) :Vasireddy, V., et al. Prog Retin Eye Res 29(3):191-207(2010)Gu, H., et al. Zhonghua Yan Ke Za Zhi 46(2):125-128(2010)DeAngelis, M.M., et al. Arch. Ophthalmol. 125(1):49-54(2007)McMahon, A., et al. Mol. Vis. 13, 258-272 (2007) :
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