Results 201 to 210 of about 6,872 (253)
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Peroxisomes, Peroxisomal Diseases, and the Hepatotoxicity Induced by Peroxisomal Metabolites
Current Drug Metabolism, 2012The group of peroxisomal disorders represents a growing number of genetically determined diseases in humans in which there is an impairment in one or more peroxisomal functions. The peroxisomal disorders are usually subdivided in two major subgroups including (1) the peroxisome biogenesis disorders (PBDs) and (2) the single peroxisomal enzyme ...
Ronald J A, Wanders +1 more
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Nature Reviews Microbiology, 2018 Peroxisomes and Peroxisomal Functions in Hyperpipecolic Acidaemia
Journal of Inherited Metabolic Disease, 1988Hyperpipecolic acidaemia (McKusick 23940) has so far been reported in only four patients (Gatfield et al., 1968; Thomas et al., 1975; Burton et al., 1981). The hallmarks of this disorder are delayed development, hepatomegaly, hypotonia, retinopathy and progressive neurological deterioration with death occurring before 2–2½ years of age.
Wanders, R. J. +6 more
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When is a peroxisome not a peroxisome?
Trends in Plant Science, 2008It is time to drop the glyoxysome name. Recent functional genomics analysis together with cell biology studies emphasize the unifying features of peroxisomes rather than their differences. Plant peroxisomes contain 300 or more proteins, the functions of which are dominated by activities related to fatty acid oxidation (>70 enzymes).
Itsara, Pracharoenwattana +1 more
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Peroxisomes in cardiomyocytes and the peroxisome / peroxisome proliferator-activated receptor-loop
Thrombosis and Haemostasis, 2015SummaryIt is well established that the heart is strongly dependent on fatty acid metabolism. In cardiomyocytes there are two distinct sites for the β-oxidisation of fatty acids: the mitochondrion and the peroxisome. Although the metabolism of these two organelles is believed to be tightly coupled, the nature of this relationship has not been fully ...
Claudia, Colasante +3 more
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Peroxisomes: Membrane events accompanying peroxisome proliferation
The International Journal of Biochemistry & Cell Biology, 2011Peroxisomes are ubiquitous organelles surrounded by a single membrane that display a variety of metabolic functions. These vary with the organism in which they occur and with environmental conditions. Peroxisomes multiply by division of existing organelles and can be formed from ER.
Opalinski, Lukasz +2 more
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Peroxisomes and Peroxisomal Disorders
1989Peroxisomes are organelles that are present in virtually all human cell types. The diameter varies between 0.15 and 1.5 µm. Morphologically peroxisomes are differentiated from mitochondria by their single membrane, electron-dense homogeneous matrix and the absence of cristae and from lysosomes which often contain vacuoles, lipids, myelin figures, or ...
Jacob Valk, Marjo S. van der Knaap
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Current Opinion in Pediatrics, 1999
Peroxisomes, subcellular organelles found in nearly all eukaryotic cells, are involved in numerous biochemical functions within the cell. There has been an increasing understanding of the genetic mechanism of the diseases of the single peroxisomal enzyme abnormalities as well as defects of peroxisome biogenesis.
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Peroxisomes, subcellular organelles found in nearly all eukaryotic cells, are involved in numerous biochemical functions within the cell. There has been an increasing understanding of the genetic mechanism of the diseases of the single peroxisomal enzyme abnormalities as well as defects of peroxisome biogenesis.
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Annual Review of Cell and Developmental Biology, 2001
▪ Abstract Fifteen years ago, we had a model of peroxisome biogenesis that involved growth and division of preexisting peroxisomes. Today, thanks to genetically tractable model organisms and Chinese hamster ovary cells, 23 PEX genes have been cloned that encode the machinery (“peroxins”) required to assemble the organelle.
P E, Purdue, P B, Lazarow
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▪ Abstract Fifteen years ago, we had a model of peroxisome biogenesis that involved growth and division of preexisting peroxisomes. Today, thanks to genetically tractable model organisms and Chinese hamster ovary cells, 23 PEX genes have been cloned that encode the machinery (“peroxins”) required to assemble the organelle.
P E, Purdue, P B, Lazarow
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The Journal of Pediatrics, 1986
Peroxisomal disorders occur more frequently and have a wider range of clinical manifestations than has been realized in the past. Precise diagnosis can be achieved with non-invasive biochemical assays and all can be diagnosed prenatally, thus providing the option of genetic counseling.
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Peroxisomal disorders occur more frequently and have a wider range of clinical manifestations than has been realized in the past. Precise diagnosis can be achieved with non-invasive biochemical assays and all can be diagnosed prenatally, thus providing the option of genetic counseling.
openaire +4 more sources