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Journal of Inherited Metabolic Disease 1994Until recently peroxisomal disorders were considered to be extremely rare and the diagnostic procedures available for postanatal and prenatal diagnosis were not widely... (Review)
Review
Until recently peroxisomal disorders were considered to be extremely rare and the diagnostic procedures available for postanatal and prenatal diagnosis were not widely known. At present, 17 human disorders are linked to peroxisomal dysfunction. The clinical, biochemical and morphological peroxisome heterogeneity described in the different diseases illustrate that only combined analysis of all the different approaches will lead to a correct diagnosis and a coherent pathophysiological model to guide ongoing research. With the study of human peroxisomal disease, advances have been gained as to the function of the peroxisome in normal and pathological conditions. Genetic analysis of peroxisome biogenesis and research on peroxisomal targeting signals are now in progress. Peroxisomal disorders are usually classified according to the degree of biochemical impairment. In this paper, a tentative classification of peroxisomal disorders will be proposed, based on the degree of biochemical abnormalities combined with new data obtained on whether or not defective peroxisome assembly is involved: (1) disorders with peroxisome assembly deficiencies; (2) disorders with single enzyme deficiencies. The clinical onset and the major symptoms of the various disorders, and the recently discovered findings are discussed.
Topics: Biological Transport; Enzymes; Genotype; Humans; Metabolism, Inborn Errors; Microbodies; Nervous System Diseases; Phenotype; Proteins
PubMed: 7967497
DOI: 10.1007/BF00711362 -
Essays in Biochemistry 1989Mammalian peroxisomes are subcellular organelles involved in the metabolism of hydrogen peroxide (oxidases, catalase), lipid anabolism (ether lipid biosynthesis) and... (Review)
Review
Mammalian peroxisomes are subcellular organelles involved in the metabolism of hydrogen peroxide (oxidases, catalase), lipid anabolism (ether lipid biosynthesis) and catabolism (oxidation of fatty acids and fatty acid derivatives), and intermediary metabolism (transaminases, dehydrogenases). Peroxisomes are formed by division, as is the case for mitochondria, but, in contrast to these organelles, they do not contain DNA. They were discovered and characterized (by biochemical and morphological techniques) later than the majority of the other cell components and specific procedures have been developed for their isolation. Functions of peroxisomes are, as a rule, shared by other cell compartments so that specific enzyme assays have also been developed. Combination of specific isolation procedures, enzyme assays and morphological analysis have resulted in our current knowledge of peroxisomal physiology which, however, has greatly benefited, as in the case of lysosomes, from the study of inborn errors of metabolism and the contribution of molecular biology. Novel enzymes and metabolic pathways have been demonstrated to exist in peroxisomes and human genetic disorders affecting one or several of these functions have been recognized.
Topics: Animals; Humans; Microbodies; Oxidation-Reduction
PubMed: 2676523
DOI: No ID Found -
The Journal of Cell Biology Jul 1985Rat liver peroxisomes were subjected to a variety of procedures intended to partially disassemble or damage them; the effects were analyzed by recentrifugation into...
Rat liver peroxisomes were subjected to a variety of procedures intended to partially disassemble or damage them; the effects were analyzed by recentrifugation into sucrose gradients, enzyme analyses, electron microscopy, and SDS PAGE. Freezing and thawing or mild sonication released some matrix proteins and produced apparently intact peroxisomal "ghosts" with crystalloid cores and some fuzzy fibrillar content. Vigorous sonication broke open the peroxisomes but the membranes remained associated with cores and fibrillar and amorphous matrix material. The density of both ghosts and more severely damaged peroxisomes was approximately 1.23. Pyrophosphate (pH 9) treatment solubilized the fibrillar content, yielding ghosts that were empty except for cores. Some matrix proteins such as catalase and thiolase readily leak from peroxisomes. Other proteins were identified that remain in mechanically damaged peroxisomes but are neither core nor membrane proteins because they can be released by pyrophosphate treatment. These constitute a class of poorly soluble matrix proteins that appear to correspond to the fibrillar material observed morphologically. All of the peroxisomal beta-oxidation enzymes are located in the matrix, but they vary greatly in how easily they leak out. Palmitoyl coenzyme A synthetase is in the membrane, based on its co-distribution with the 22-kilodalton integral membrane polypeptide.
Topics: Animals; Cell Compartmentation; Cell Fractionation; Diphosphates; Female; Freezing; Liver; Microbodies; Microscopy, Electron; Polyethylene Glycols; Proteins; Rats; Solubility; Sonication
PubMed: 2989301
DOI: 10.1083/jcb.101.1.294 -
Sub-cellular Biochemistry 1994
Review
Topics: Animals; Humans; Intracellular Membranes; Microbodies
PubMed: 8146884
DOI: 10.1007/978-1-4615-2401-4_8 -
Archives of Biochemistry and Biophysics Mar 1975
Topics: Cell Fractionation; Centrifugation, Density Gradient; Choline; Darkness; Microbodies; Organoids; Phosphatidylcholines; Plant Physiological Phenomena; Plants; Subcellular Fractions; Time Factors
PubMed: 1130799
DOI: 10.1016/0003-9861(75)90439-7 -
Biochimie 1993This article summarizes our current knowledge of the metabolic pathways present in mammalian peroxisomes. Emphasis is placed on those aspects that are not covered by... (Review)
Review
This article summarizes our current knowledge of the metabolic pathways present in mammalian peroxisomes. Emphasis is placed on those aspects that are not covered by other articles in this issue: peroxisomal enzyme content and topology; the peroxisomal beta-oxidation system; substrates of peroxisomal beta-oxidation such as very-long-chain fatty acids, branched fatty acids, dicarboxylic fatty acids, prostaglandins and xenobiotics; the role of peroxisomes in the metabolism of purines, polyamines, amino acids, glyoxylate and reactive oxygen products such as hydrogen peroxide, superoxide anions and epoxides.
Topics: Amino Acids; Animals; Fatty Acids; Glyoxylates; Humans; Mammals; Microbodies; Oxidation-Reduction; Oxygen; Reactive Oxygen Species
PubMed: 8507676
DOI: 10.1016/0300-9084(93)90072-z -
Endeavour 1996Peroxisomes are subcellular organelles that are present in all eukaryotic organisms. These organelles are the focus of much contemporary interest among cellular and... (Review)
Review
Peroxisomes are subcellular organelles that are present in all eukaryotic organisms. These organelles are the focus of much contemporary interest among cellular and medical biologists--an interest which coincides with the realization of their vital role in higher organisms, their unique metabolic and biogenetic characteristics, and their widespread involvement in genetic and degenerative disease. This article reviews some of the major recent developments in peroxisome biology.
Topics: Animals; Biology; Free Radicals; Genetic Diseases, Inborn; Humans; Microbodies; Oxidation-Reduction; Reactive Oxygen Species
PubMed: 8690001
DOI: 10.1016/0160-9327(96)10009-0 -
Annual Review of Biochemistry 1981
Review
Topics: Alcohols; Animals; Cell Fractionation; Fatty Acids; Glyceric Acids; Glycolates; Glyoxylates; Liver; Microbodies; Organoids; Oxidation-Reduction; Plants
PubMed: 7023357
DOI: 10.1146/annurev.bi.50.070181.001025 -
Archives of Microbiology Feb 1977Microbodies appearing abundantly in n-alkane-grown cells of Candida tropicalis pK 233 were isolated by means of sucrose density gradient centrifugation. Electron...
Microbodies appearing abundantly in n-alkane-grown cells of Candida tropicalis pK 233 were isolated by means of sucrose density gradient centrifugation. Electron microscopical observation showed that the microbodies isolated were intact. Localization of catalase and D-amino acid oxidase in the isolated microbodies was confirmed. Isocitrate lyase, melate synthase and NADP-linked isocitrate dehydrogenase were also located in the microbody, but malate dehydrogenase, citrate synthase, aconitase and NAD-linked isocitrate dehydrogenase were not. Neither cytochrome P-450 not NADPH-cytochrome c reductase, the components involved in the n-alkane hydroxylation system of the yeast, were detected in the microbody fraction.
Topics: Alkanes; Candida; Catalase; Cell Fractionation; Centrifugation, Density Gradient; Glyoxylates; Microbodies; Microscopy, Electron; Organoids
PubMed: 843163
DOI: 10.1007/BF00446647 -
Molecular and Cellular Biochemistry Dec 1996The interactions between the omega-3 unsaturated fatty acids and peroxisomal function have been reviewed, in order to update and integrate knowledge in this area.... (Review)
Review
The interactions between the omega-3 unsaturated fatty acids and peroxisomal function have been reviewed, in order to update and integrate knowledge in this area. Following a brief retrospective of the major clinical involvements of these fatty acids, the participation of the peroxisome in their metabolism has been appraised-the peroxisome being shown to exert a major influence on both the synthesis and degradation of the omega-3 fatty acids, with these effects flowing on to the widespread physiological implications of the derivative eicosanoids. Interactions between the omega-3 and omega-6 families of fatty acids have been discussed, as have the interdependent phenomena of peroxisome proliferation, membrane remodelling and cellular signalling. Amongst the signalling involvements covered were those of steroid hormone receptor superfamily, the phosphatidylcholine cycle, and the regulatory influences of oxygen free radicals. Comment has also been included on the separate biological roles of the individual omega-3 fatty acids, their influence on differential gene function, and on the molecular mechanisms of their pharmacological effects. It is concluded that the peroxisome is intimately involved in directing the metabolism and physiological influence of the omega-3 unsaturated fatty acids, and that this organelle merits much greater emphasis in future research aimed at unravelling the profound biological effects of these unique and multipotent compounds.
Topics: Animals; Eicosanoids; Fatty Acids, Omega-3; Fatty Acids, Omega-6; Fatty Acids, Unsaturated; Gene Expression; Humans; Microbodies
PubMed: 8979256
DOI: 10.1007/BF00229469