Results 211 to 220 of about 6,872 (253)
Some of the next articles are maybe not open access.
2014
Peroxisomal diseases are a group of genetically and clinically divergent diseases due to defects in either the biogenesis of peroxisomes (group 1) or one of its metabolic functions (group 2). Zellweger syndrome and X-linked adrenoleukodystrophy (XALD) are the prototypes for group 1 and 2, respectively.
openaire +4 more sources
Peroxisomal diseases are a group of genetically and clinically divergent diseases due to defects in either the biogenesis of peroxisomes (group 1) or one of its metabolic functions (group 2). Zellweger syndrome and X-linked adrenoleukodystrophy (XALD) are the prototypes for group 1 and 2, respectively.
openaire +4 more sources
Biochemistry and Cell Biology, 1991
The concept that there are human disease states that are associated with abnormal peroxisomal function is of recent origin. This is due in part to the relatively recent discovery of the organelle itself by de Duve in 1983, and to the earlier belief that it was a vestigial structure in mammals.
H W, Moser, A, Bergin, D, Cornblath
openaire +2 more sources
The concept that there are human disease states that are associated with abnormal peroxisomal function is of recent origin. This is due in part to the relatively recent discovery of the organelle itself by de Duve in 1983, and to the earlier belief that it was a vestigial structure in mammals.
H W, Moser, A, Bergin, D, Cornblath
openaire +2 more sources
2013
The peroxisomal disorders represent a group of genetic diseases in man in which there is an impairment in one or more peroxisomal functions. The peroxisomal disorders are subdivided into three subgroups comprising: (1) the peroxisome biogenesis disorders (PBDs); (2) the single peroxisomal (enzyme-) protein deficiencies; and (3) the single peroxisomal ...
Patrick, Aubourg, Ronald, Wanders
openaire +2 more sources
The peroxisomal disorders represent a group of genetic diseases in man in which there is an impairment in one or more peroxisomal functions. The peroxisomal disorders are subdivided into three subgroups comprising: (1) the peroxisome biogenesis disorders (PBDs); (2) the single peroxisomal (enzyme-) protein deficiencies; and (3) the single peroxisomal ...
Patrick, Aubourg, Ronald, Wanders
openaire +2 more sources
Hospital Practice, 1985
It is now recognized that absence of normal peroxisomal function is associated with far-reaching and devastating consequences. A group of diverse genetic disorders in which either defective formation of the peroxisome or deficiency of one or more of its constituent enzymes is the central pathogenetic feature has recently been described. Three principal
H W, Moser, S L, Goldfischer
openaire +2 more sources
It is now recognized that absence of normal peroxisomal function is associated with far-reaching and devastating consequences. A group of diverse genetic disorders in which either defective formation of the peroxisome or deficiency of one or more of its constituent enzymes is the central pathogenetic feature has recently been described. Three principal
H W, Moser, S L, Goldfischer
openaire +2 more sources
Peroxisomes and Photomorphogenesis
2013In higher plants, light-grown seedlings exhibit photomorphogenesis, a developmental program controlled by a complex web of interactions between photoreceptors, central repressors, and downstream effectors that leads to changes in gene expression and physiological changes. Light induces peroxisomal proliferation through a phytochrome A-mediated pathway,
Navneet, Kaur, Jiying, Li, Jianping, Hu
openaire +2 more sources
Trends in Cell Biology, 2007
Peroxisomes are a dynamic compartment in almost all eukaryotic cells and have diverse metabolic roles in response to environmental changes and cellular demands. The accompanying changes in enzyme content or abundance of peroxisomes are accomplished by dynamically operating membrane- and matrix-protein transport machineries. This review discusses recent
Harald W, Platta, Ralf, Erdmann
openaire +2 more sources
Peroxisomes are a dynamic compartment in almost all eukaryotic cells and have diverse metabolic roles in response to environmental changes and cellular demands. The accompanying changes in enzyme content or abundance of peroxisomes are accomplished by dynamically operating membrane- and matrix-protein transport machineries. This review discusses recent
Harald W, Platta, Ralf, Erdmann
openaire +2 more sources
Seminars in Neonatology, 2002
Peroxisomes are subcellular organelles catalyzing a number of indispensable functions in cellular metabolism. The importance of peroxisomes is stressed by the existence of an expanding number of genetic diseases in which there is an impairment of one or more peroxisomal functions.
Baumgartner, Matthias R +1 more
openaire +2 more sources
Peroxisomes are subcellular organelles catalyzing a number of indispensable functions in cellular metabolism. The importance of peroxisomes is stressed by the existence of an expanding number of genetic diseases in which there is an impairment of one or more peroxisomal functions.
Baumgartner, Matthias R +1 more
openaire +2 more sources
The Cytoplasm: Peroxisomes and Peroxisomal Diseases
2015Peroxisomes are ubiquitous organelles that contain catalase and oxidative enzymes producing H2O2. Depending on cell type, their number, shape, and size vary. By electron microscopy, peroxisomes have a single membrane that encloses a dense matrix that contains a crystalloid core in some species (e.g., rat hepatocytes) but not in others (e.g., human ...
Margit Pavelka, Jürgen Roth
openaire +1 more source
Brain and Development, 1987
The different types of adrenoleukodystrophy are considered with their clinical and biochemical features, particularly the excess of very-long-chain fatty acids. Then other conditions which show this latter finding are described, including the Zellweger cerebrohepatorenal syndrome, hyperpipecolic acidemia and Refsum disease.
openaire +2 more sources
The different types of adrenoleukodystrophy are considered with their clinical and biochemical features, particularly the excess of very-long-chain fatty acids. Then other conditions which show this latter finding are described, including the Zellweger cerebrohepatorenal syndrome, hyperpipecolic acidemia and Refsum disease.
openaire +2 more sources
Gluconeogenesis and the peroxisome
Molecular and Cellular Biochemistry, 1997In this article, the capabilities of peroxisomal involvement in the gluconeogenetic processes of vertebrate animals are reviewed in the light of recent findings on peroxisomal metabolism and proliferation. It is demonstrated that the participation of this organelle affords the potential of alternative pathways for the conversion of triacylglycerols to ...
openaire +2 more sources