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Rabbit Anti-ELOVL4 Recombinant Antibody (EG1097) (CBMAB-EN1293-LY)

The product is antibody recognizes ELOVL4. The antibody EG1097 immunoassay techniques such as: WB: 1:500~1:1000 ELISA: 1:40000.
See all ELOVL4 antibodies

Summary

Host Animal
Rabbit
Specificity
Human, Mouse
Clone
EG1097
Antibody Isotype
IgG
Application
WB: 1:500~1:1000 ELISA: 1:40000

Basic Information

Immunogen
The antibody was produced against synthesized peptide derived from internal of human ELOVL4.
Specificity
Human, Mouse
Antibody Isotype
IgG
Application Notes
The COA includes recommended starting dilutions, optimal dilutions should be determined by the end user.

Formulations & Storage [For reference only, actual COA shall prevail!]

Format
Liquid
Storage
Store at +4°C short term (1-2 weeks). Aliquot and store at -20°C long term. Avoid repeated freezethaw cycles.

Target

Full Name
ELOVL Fatty Acid Elongase 4
Introduction
This gene encodes a membrane-bound protein which is a member of the ELO family, proteins which participate in the biosynthesis of fatty acids. Consistent with the expression of the encoded protein in photoreceptor cells of the retina, mutations and small deletions in this gene are associated with Stargardt-like macular dystrophy (STGD3) and autosomal dominant Stargardt-like macular dystrophy (ADMD), also referred to as autosomal dominant atrophic macular degeneration. [provided by RefSeq, Jul 2008]
Entrez Gene ID
Human6785
Mouse83603
UniProt ID
HumanQ9GZR5
MouseQ9EQC4
Alternative Names
ELOVL Fatty Acid Elongase 4; Elongation Of Very Long Chain Fatty Acids (FEN1/Elo2, SUR4/Elo3, Yeast)-Like 4; Very Long Chain 3-Ketoacyl-CoA Synthase 4; Very Long Chain 3-Oxoacyl-CoA Synthase 4; 3-Keto Acyl-CoA Synthase ELOVL4; Cancer/Testis Antigen 118; ELOVL FA Elongase 4; Elongation Of Very Long Chain Fatty Acids Protein 4; Spinocerebellar Ataxia 34;
Research Area
Catalyzes the first and rate-limiting reaction of the four reactions 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 catalyzes the synthesis of very long chain saturated (VLC-SFA) and polyunsaturated (PUFA) fatty acids 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.
Biological Process
Fatty acid biosynthetic process Source: UniProtKB
Fatty acid elongation, monounsaturated fatty acid Source: GO_Central
Fatty acid elongation, polyunsaturated fatty acid Source: UniProtKB
Fatty acid elongation, saturated fatty acid Source: UniProtKB
Long-chain fatty-acyl-CoA biosynthetic process Source: UniProtKB-UniRule
Sphingolipid biosynthetic process Source: GO_Central
Unsaturated fatty acid biosynthetic process Source: UniProtKB-UniRule
Very long-chain fatty acid biosynthetic process Source: UniProtKB
Cellular Location
Endoplasmic reticulum membrane
Involvement in disease
Stargardt disease 3 (STGD3):
A common hereditary macular degeneration. It is characterized by decreased central vision, atrophy of the macula and underlying retinal pigment epithelium, and frequent presence of prominent flecks in the posterior pole of the retina.
Ichthyosis, spastic quadriplegia, and mental retardation (ISQMR):
A severe autosomal recessive disorder characterized by ichthyosis apparent from birth, profound psychomotor retardation with essentially no development, spastic quadriplegia, and seizures.
Spinocerebellar ataxia 34 (SCA34):
A form of spinocerebellar ataxia, a clinically and genetically heterogeneous group of cerebellar disorders. Patients show progressive incoordination of gait and often poor coordination of hands, speech and eye movements, due to degeneration of the cerebellum with variable involvement of the brainstem and spinal cord. SCA34 is an autosomal dominant form characterized by the association of progressive cerebellar ataxia with erythrokeratodermia variabilis.
PTM
N-glycosylated.

Luo, J., Monroig, Ó., Liao, K., Ribes-Navarro, A., Navarro, J. C., Zhu, T., ... & Jin, M. (2021). Biosynthesis of LC-PUFAs and VLC-PUFAs in Pampus argenteus: Characterization of Elovl4 elongases and regulation under acute salinity. Journal of Agricultural and Food Chemistry, 69(3), 932-944.

Yeboah, G. K., Lobanova, E. S., Brush, R. S., & Agbaga, M. P. (2021). Very long chain fatty acid-containing lipids: A decade of novel insights from the study of ELOVL4. Journal of Lipid Research, 62.

Rugolo, F., Bazan, N. G., Calandria, J., Jun, B., Raschellà, G., Melino, G., & Agostini, M. (2021). The expression of ELOVL4, repressed by MYCN, defines neuroblastoma patients with good outcome. Oncogene, 40(38), 5741-5751.

He, Y., Phan, K., Bhatia, S., Pickford, R., Fu, Y., Yang, Y., ... & Kim, W. S. (2021). Increased VLCFA-lipids and ELOVL4 underlie neurodegeneration in frontotemporal dementia. Scientific reports, 11(1), 1-11.

Betancor, M. B., Oboh, A., Ortega, A., Mourente, G., Navarro, J. C., de la Gándara, F., ... & Monroig, Ó. (2020). Molecular and functional characterisation of a putative elovl4 gene and its expression in response to dietary fatty acid profile in Atlantic bluefin tuna (Thunnus thynnus). Comparative Biochemistry and Physiology Part B: Biochemistry and Molecular Biology, 240, 110372.

Ting, S. Y., Janaranjani, M., Merosha, P., Sam, K. K., Wong, S. C., Goh, P. T., ... & Chong Shu-Chien, A. (2020). Two elongases, Elovl4 and Elovl6, fulfill the elongation routes of the LC-PUFA biosynthesis pathway in the orange mud crab (Scylla olivacea). Journal of agricultural and food chemistry, 68(14), 4116-4130.

Deák, F., Anderson, R. E., Fessler, J. L., & Sherry, D. M. (2019). Novel cellular functions of very long chain-fatty acids: insight from ELOVL4 mutations. Frontiers in cellular neuroscience, 13, 428.

Hopiavuori, B. R., Anderson, R. E., & Agbaga, M. P. (2019). ELOVL4: Very long-chain fatty acids serve an eclectic role in mammalian health and function. Progress in retinal and eye research, 69, 137-158.

Donato, L., Scimone, C., Rinaldi, C., Aragona, P., Briuglia, S., D'Ascola, A., ... & Sidoti, A. (2018). Stargardt phenotype associated with two ELOVL4 promoter variants and ELOVL4 downregulation: new possible perspective to etiopathogenesis?. Investigative ophthalmology & visual science, 59(2), 843-857.

Bourque, P. R., Warman-Chardon, J., Lelli, D. A., LaBerge, L., Kirshen, C., Bradshaw, S. H., ... & Boycott, K. M. (2018). Novel ELOVL4 mutation associated with erythrokeratodermia and spinocerebellar ataxia (SCA 34). Neurology Genetics, 4(4).

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For research use only. Not intended for any clinical use.

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