Long description 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.
Antibody come from Synthetic peptide derived from the human MiTF protein
Other description Provided as solution in phosphate buffered saline with 0.08% sodium azide. Antigen Immunoaffiinity Purification
Antigen-antibody binding interaction Rabbit anti ELOVL4 Antibody
Antibody is raised in Rabbit
Antibody's reacts with Human
Antibody's reacts with these species This antibody doesn't cross react with other species
Antibody's specificity No Data Available
Application Western Blot
Antibody's suited for Antibody can be used for Western blotting (1-5 µg/ml). Optimal concentration should be evaluated by serial dilutions.
Relevant references 1. Weilbaecher KN, et. al. Age-resolving osteopetrosis: a rat model implicating microphthalmia and the related transcription factor TFE3. J. Exp.Med. 1998, 187: 775-785_x000B__x000B_2. Hemesath P, et. al. MAP kinase links the transcription factor microphthalmia to c-Kit signalling in melanocytes. Nature. 1998, 391:298-301
Protein number see ncbi
Warnings This product is intended FOR RESEARCH USE ONLY, and FOR TESTS IN VITRO, not for use in diagnostic or therapeutic procedures involving humans or animals. This datasheet is as accurate as reasonably achievable, but Nordic-MUbio accepts no liability for any inaccuracies or omissions in this information.
About Rabbits are used for polyclonal antibody production by nordc. Rabbit antibodies are very stable and can be stored for several days at room temperature. nordc adds sodium azide and glycerol to enhance the stability of the rabbit polyclonal antibodies. Anti-human, anti mouse antibodies to highly immunogenic selected peptide sequences are" monoclonal like" since the epitope to which they are directed is less than 35 amino acids long.