ELOVL4
Reactivité: Humain
ELISA
Hôte: Lapin
Polyclonal
Biotin
Indications d'application
Western blot (1-5 μg/mL starting dilution). Other applications not tested. Optimal dilutions are dependent on conditions and should be determined by the user.
Restrictions
For Research Use only
Buffer
Phosphate buffered saline with 0.08 % sodium azide
Agent conservateur
Sodium azide
Précaution d'utilisation
This product contains sodium azide: a POISONOUS AND HAZARDOUS SUBSTANCE which should be handled by trained staff only.
Conseil sur la manipulation
Avoid repeated freezing and thawing.
Stock
-20 °C
Stockage commentaire
Store the product (in aliquots) at -20 °C. Can be shipped at 2 - 8 °C.
anticorps elovl4, anticorps MGC122553, anticorps ADMD, anticorps CT118, anticorps ISQMR, anticorps STGD2, anticorps STGD3, anticorps elongation of very long chain fatty acids protein 4, anticorps ELOVL fatty acid elongase 4, anticorps elongation of very long chain fatty acids (FEN1/Elo2, SUR4/Elo3, yeast)-like 4, anticorps CpipJ_CPIJ002114, anticorps CpipJ_CPIJ003687, anticorps CpipJ_CPIJ011025, anticorps CpipJ_CPIJ013819, anticorps ELOVL4, anticorps elovl4, anticorps Elovl4
Sujet
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.Synonyms: 3-keto acyl-CoA synthase ELOVL4, Elongation of very long chain fatty acids protein 4