Hetero-oligomeric interactions of an ELOVL4 mutant protein: implications in the molecular mechanism of Stargardt-3 macular dystrophy
暂无分享,去创建一个
A. Kihara | Y. Igarashi | Kensuke Abe | Tatsuro Naganuma | Y. Ohno | A. Okuda | M. Yamagata | Yusuke Ohno
[1] H. Konishi,et al. Characterization of HACD1 K64Q mutant found in arrhythmogenic right ventricular dysplasia patients. , 2010, Journal of biochemistry.
[2] Shota Suto,et al. ELOVL1 production of C24 acyl-CoAs is linked to C24 sphingolipid synthesis , 2010, Proceedings of the National Academy of Sciences.
[3] M. Agbaga,et al. Retinal very long-chain PUFAs: new insights from studies on ELOVL4 protein , 2010, Journal of Lipid Research.
[4] M. Agbaga,et al. Role of Stargardt-3 macular dystrophy protein (ELOVL4) in the biosynthesis of very long chain fatty acids , 2008, Proceedings of the National Academy of Sciences.
[5] A. Kihara,et al. Characterization of four mammalian 3‐hydroxyacyl‐CoA dehydratases involved in very long‐chain fatty acid synthesis , 2008, FEBS letters.
[6] A. Kihara,et al. Membrane Topology and Essential Amino Acid Residues of Phs1, a 3-Hydroxyacyl-CoA Dehydratase Involved in Very Long-chain Fatty Acid Elongation* , 2008, Journal of Biological Chemistry.
[7] A. Woods,et al. A Stargardt disease‐3 mutation in the mouse Elovl4 gene causes retinal deficiency of C32–C36 acyl phosphatidylcholines , 2007, FEBS letters.
[8] J. Weissman,et al. A Molecular Caliper Mechanism for Determining Very Long-Chain Fatty Acid Length , 2007, Cell.
[9] Radha Ayyagari,et al. Loss of functional ELOVL4 depletes very long-chain fatty acids (≥C28) and the unique ω-O-acylceramides in skin leading to neonatal death , 2007 .
[10] A. Edwards,et al. Retinal pathology and skin barrier defect in mice carrying a Stargardt disease-3 mutation in elongase of very long chain fatty acids-4 , 2007, Molecular vision.
[11] C. Deng,et al. Depletion of ceramides with very long chain fatty acids causes defective skin permeability barrier function, and neonatal lethality in ELOVL4 deficient mice , 2007, International journal of biological sciences.
[12] A. A. Spector,et al. Essentiality of fatty acids , 2007, Lipids.
[13] P. Sieving,et al. Elovl4 5-bp-deletion knock-in mice develop progressive photoreceptor degeneration. , 2006, Investigative ophthalmology & visual science.
[14] R. Westerberg,et al. Fatty acid elongases in mammals: their regulation and roles in metabolism. , 2006, Progress in lipid research.
[15] R. Molday,et al. Dominant Negative Mechanism Underlies Autosomal Dominant Stargardt-like Macular Dystrophy Linked to Mutations in ELOVL4* , 2005, Journal of Biological Chemistry.
[16] P. Sieving,et al. Stargardt-like macular dystrophy protein ELOVL4 exerts a dominant negative effect by recruiting wild-type protein into aggresomes. , 2005, Molecular vision.
[17] Yu Zhao,et al. Loss of ER retention and sequestration of the wild-type ELOVL4 by Stargardt disease dominant negative mutants. , 2005, Molecular vision.
[18] P. Sieving,et al. Characterization of mouse orthologue of ELOVL4: genomic organization and spatial and temporal expression. , 2004, Genomics.
[19] P. Sieving,et al. Atrophic macular degeneration mutations in ELOVL4 result in the intracellular misrouting of the protein. , 2004, Genomics.
[20] H. Sprecher,et al. Elongation of long-chain fatty acids. , 2004, Progress in lipid research.
[21] A. Kihara,et al. Sphingosine-1-phosphate Lyase Is Involved in the Differentiation of F9 Embryonal Carcinoma Cells to Primitive Endoderm* , 2003, The Journal of Biological Chemistry.
[22] J. Horton,et al. Identification of Two Mammalian Reductases Involved in the Two-carbon Fatty Acyl Elongation Cascade* , 2003, The Journal of Biological Chemistry.
[23] C. J. Lewis,et al. Identification and expression of mammalian long-chain PUFA elongation enzymes , 2002, Lipids.
[24] J. Warrington,et al. Identification of a Mammalian Long Chain Fatty Acyl Elongase Regulated by Sterol Regulatory Element-binding Proteins* , 2001, The Journal of Biological Chemistry.
[25] A. Edwards,et al. Autosomal dominant Stargardt-like macular dystrophy. , 2001, Survey of ophthalmology.
[26] M. Metzker,et al. A 5-bp deletion in ELOVL4 is associated with two related forms of autosomal dominant macular dystrophy , 2001, Nature Genetics.
[27] J. Dorado,et al. Cloning of a human cDNA encoding a novel enzyme involved in the elongation of long-chain polyunsaturated fatty acids. , 2000, The Biochemical journal.
[28] A. Edwards,et al. Autosomal dominant Stargardt-like macular dystrophy: I. Clinical characterization, longitudinal follow-up, and evidence for a common ancestry in families linked to chromosome 6q14. , 1999, American journal of ophthalmology.
[29] Stuart Smith. The animal fatty acid synthase: one gene, one polypeptide, seven enzymes , 1994, FASEB journal : official publication of the Federation of American Societies for Experimental Biology.