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Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism

Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism

http://www.wikidata.org/entity/Q34116305

about

Autophagic degradation of peroxisomes in mammals Brain Lipotoxicity of Phytanic Acid and Very Long-chain Fatty Acids. Harmful Cellular/Mitochondrial Activities in Refsum Disease and X-Linked Adrenoleukodystrophy Metabolic Interplay between Peroxisomes and Other Subcellular Organelles Including Mitochondria and the Endoplasmic Reticulum Peroxisomes in brain development and function Zellweger spectrum disorders: clinical overview and management approach Hypoxia signaling pathways: modulators of oxygen-related organelles A review of odd-chain fatty acid metabolism and the role of pentadecanoic Acid (c15:0) and heptadecanoic Acid (c17:0) in health and disease Cre-mediated stress affects sirtuin expression levels, peroxisome biogenesis and metabolism, antioxidant and proinflammatory signaling pathways Redox interplay between mitochondria and peroxisomes Regulation of peroxisomal lipid metabolism by catalytic activity of tumor suppressor H-rev107 Oxidative status interactome map: towards novel approaches in experiment planning, data analysis, diagnostics and therapy.Peroxisomal biogenesis is genetically and biochemically linked to carbohydrate metabolism in Drosophila and mouse.Gene expression changes consistent with neuroAIDS and impaired working memory in HIV-1 transgenic rats.Acyl-CoA thioesterase 9 (ACOT9) in mouse may provide a novel link between fatty acid and amino acid metabolism in mitochondria.Lipid metabolism in myelinating glial cells: lessons from human inherited disorders and mouse models.Glial β-oxidation regulates Drosophila energy metabolism.The beta-3 adrenergic agonist (CL-316,243) restores the expression of down-regulated fatty acid oxidation genes in type 2 diabetic mice The peroxisomal protein import machinery displays a preference for monomeric substrates.Interaction of Phospholipase A/Acyltransferase-3 with Pex19p: A POSSIBLE INVOLVEMENT IN THE DOWN-REGULATION OF PEROXISOMES Peroxisomal L-bifunctional enzyme (Ehhadh) is essential for the production of medium-chain dicarboxylic acids The metabolomics of asthma control: a promising link between genetics and disease Export-deficient monoubiquitinated PEX5 triggers peroxisome removal in SV40 large T antigen-transformed mouse embryonic fibroblasts.Integration of peroxisomes into an endomembrane system that governs cellular aging.Peroxisome degradation in mammals: mechanisms of action, recent advances, and perspectives Phytanic acid activates NADPH oxidase through transactivation of epidermal growth factor receptor in vascular smooth muscle cells.The important role of biochemical and functional studies in the diagnostics of peroxisomal disorders Peroxisome deficient invertebrate and vertebrate animal models Statins Increase Mitochondrial and Peroxisomal Fatty Acid Oxidation in the Liver and Prevent Non-Alcoholic Steatohepatitis in Mice.A family of membrane-shaping proteins at ER subdomains regulates pre-peroxisomal vesicle biogenesis.Developmental roles of D-bifunctional protein-A zebrafish model of peroxisome dysfunction.Odd Chain Fatty Acids; New Insights of the Relationship Between the Gut Microbiota, Dietary Intake, Biosynthesis and Glucose Intolerance.Emerging role of the endoplasmic reticulum in peroxisome biogenesis.Mitochondrial free radical theory of aging: who moved my premise?Peroxisome-mitochondria interplay and disease.Peroxisomal Disorders: A Review on Cerebellar Pathologies.De novo peroxisome biogenesis: Evolving concepts and conundrums.Candidate genes for male and female reproductive traits in Canchim beef cattle Functional characterisation of peroxisomal β-oxidation disorders in fibroblasts using lipidomics.The role of lipid droplet formation in the protection of unsaturated fatty acids against palmitic acid induced lipotoxicity to rat insulin-producing cells.Investigating the genetic regulation of the expression of 63 lipid metabolism genes in the pig skeletal muscle.

P2860

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P2860

Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism

http://www.wikidata.org/entity/Q34116305

description

2010 nî lūn-bûn

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2010 թուականի Յունիսին հրատարակուած գիտական յօդուած

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2010 թվականի հունիսին հրատարակված գիտական հոդված

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2010年の論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年論文

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2010年论文

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name

Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism

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Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism

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Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism

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type

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Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism

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Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism

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Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism

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Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism

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Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism

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Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism

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Journal of Lipid Research

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Biochemistry and genetics of inherited disorders of peroxisomal fatty acid metabolism

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Paul P Van Veldhoven

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P2860

Identification and characterization of human PMP34, a protein closely related to the peroxisomal integral membrane protein PMP47 of Candida boidinii

Human very-long-chain acyl-CoA synthetase: cloning, topography, and relevance to branched-chain fatty acid metabolism

Molecular cloning and expression of human carnitine octanoyltransferase: evidence for its role in the peroxisomal beta-oxidation of branched-chain fatty acids

Purification, molecular cloning, and expression of 2-hydroxyphytanoyl-CoA lyase, a peroxisomal thiamine pyrophosphate-dependent enzyme that catalyzes the carbon-carbon bond cleavage during alpha-oxidation of 3-methyl-branched fatty acids

Mutations in the gene encoding peroxisomal alpha-methylacyl-CoA racemase cause adult-onset sensory motor neuropathy

Phytanoyl-CoA hydroxylase: recognition of 3-methyl-branched acyl-coAs and requirement for GTP or ATP and Mg(2+) in addition to its known hydroxylation cofactors

Molecular cloning and expression of mammalian peroxisomal trans-2-enoyl-coenzyme A reductase cDNAs

Very long-chain acyl-CoA synthetases. Human "bubblegum" represents a new family of proteins capable of activating very long-chain fatty acids

Characterisation of human peroxisomal 2,4-dienoyl-CoA reductase

Human peroxisomal 3-oxoacyl-coenzyme A thiolase deficiency

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10.1194/JLR.R005959

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10

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51

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