Exalpha Biologicals, Inc.

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Exalpha Biologicals, Inc.

ELOVL4

  • Product Code: X2381P
  • Size: 100 µg
  • Price (USD): $249

Cat #

X2381P		 Quantity:      

Data Sheet

Product Name

ELOVL4

Synonyms

Elongation of very long chain fatty acids protein 4, ELOV4

Host/Source

Rabbit

Isotype

IgG

Product Type

Antigen Immunoaffinity Purified Polyclonal

Reactivity

Human

Applications

Western Blot

Purification

Antigen Immunoaffiinity Purification

Size

100 µg

Price (USD)

$249

Background

Mutations in elongation of very long-chain fatty acid-4 (ELOVL4) are associated with autosomal dominant Stargardt-like macular degeneration (STGD3), with a five base-pair (5 bp) deletion mutation resulting in the loss of 51 carboxy-terminal amino acids and truncation of the protein. Elovl4 is expressed in the retina and only a limited number of mammalian other tissues, including skin, with unknown function. In a mouse model with the 5-bp deletion in the Elovl4 gene, mice in the heterozygous state (Elovl4(+/del)) demonstrate progressive photoreceptor degeneration. Homozygous mice (Elovl4(del/del)) display scaly, wrinkled skin, have severely compromised epidermal permeability barrier function, and die within a few hours after birth. Lipid analyses of epidermis from Elovl4(del/del) mice show a decrease in very long-chain fatty acids (VLFAs) in both the ceramide/glucosylceramide and the free fatty-acid fractions. ELOVL4 is required for generating VLFA critical for epidermal barrier function.

Immunogen

Synthetic peptdide derived from the human ELOV4 protein

Positive Control

Normal human testis. Expressed in the retina and at much lower level in the brain.

Formulation

Provided as solution in phosphate buffered saline with 0.08% sodium azide

Customer Storage

Product should be stored at -20ºC. Aliquot to avoid freeze/thaw cycles

Target Molecular Weight

36829

Product Image

Image Legend

Immunohistochemical staining of normal human testis tissue using ELOV4 antibody (Cat. No. X2381P) at 15 µg/ml.

Database Links:

SwissProtQ9GZR5Human

References

1:Kobayashi T, et al. ELOVL2 overexpression enhances triacylglycerol synthesis in 3T3-L1 and F442A cells. FEBS Lett. 2007 Jul 10;581(17):3157-63. Epub 2007 Jun 11. 2: DeAngelis MM, et al. Cigarette smoking, CFH, APOE, ELOVL4, and risk of neovascular age-related macular degeneration. Arch Ophthalmol. 2007 Jan;125(1):49-54. 3: Vasireddy V, et al. Loss of functional ELOVL4 depletes very long-chain fatty acids (> or =C28) and the unique omega-O-acylceramides in skin leading to neonatal death. Hum Mol Genet. 2007 Mar 1;16(5):471-82. Epub 2007 Jan 5. 4: Sridharan S, et al. X-ray crystal structure of Mycobacterium tuberculosis beta-ketoacyl acyl carrier protein synthase II (mtKasB). J Mol Biol. 2007 Feb 16;366(2):469-80. Epub 2006 Nov 7. 5: Seitsonen S, et al. Analysis of variants in the complement factor H, the elongation of very long chain fatty acids-like 4 and the hemicentin 1 genes of age-related macular degeneration in the Finnish population. Mol Vis. 2006 Jul 20;12:796-801. 6: Jakobsson A, et al. Fatty acid elongases in mammals: their regulation and roles in metabolism. Prog Lipid Res. 2006 May;45(3):237-49. Epub 2006 Mar 6. Review. 7: Pleite R, et al. Inhibitors of fatty acid biosynthesis in sunflower seeds. J Plant Physiol. 2006 Sep;163(9):885-94. Epub 2006 Feb 28. 8: Kajikawa M, et al. Isolation and functional characterization of fatty acid delta5-elongase gene from the liverwort Marchantia polymorpha L. FEBS Lett. 2006 Jan 9;580(1):149-54. Epub 2005 Dec 6. 9: Koo AJ, et al. Identification of a plastid acyl-acyl carrier protein synthetase in Arabidopsis and its role in the activation and elongation of exogenous fatty acids. Plant J. 2005 Nov;44(4):620-32. 10: Zhang K., et al. A 5-bp deletion in ELOVL4 is associated with two related forms of autosomal dominant macular dystrophy.; Nat. Genet. 27:89-93(2001). 11: Edwards A.O., et al; A novel gene for autosomal dominant Stargardt-like macular dystrophy with homology to the SUR4 protein family.; Invest. Ophthalmol. Vis. Sci. 42:2652-2663(2001).