Results 271 to 280 of about 43,085 (313)
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2005
Peroxisomes, one of single membrane-bound organelles, are present ubiquitously in eukaryotic cells. They were originally identified as organelles for production of hydrogen peroxide, the degradation of its hydrogen peroxide, and metabolism of fatty acids, which are functions common to almost all the organisms.
Shoji, Mano, Mikio, Nishimura
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Peroxisomes, one of single membrane-bound organelles, are present ubiquitously in eukaryotic cells. They were originally identified as organelles for production of hydrogen peroxide, the degradation of its hydrogen peroxide, and metabolism of fatty acids, which are functions common to almost all the organisms.
Shoji, Mano, Mikio, Nishimura
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BioEssays, 1997
AbstractPeroxisomes are eukaryotic organelles that are the subcellular location of important metabolic reactions. In humans, defects in the organelle's function are often lethal. Yet, relative to other organelles, little is known about how cells maintain and propagate peroxisomes or how they direct specific sets of newly synthesized proteins to these ...
H R, Waterham, J M, Cregg
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AbstractPeroxisomes are eukaryotic organelles that are the subcellular location of important metabolic reactions. In humans, defects in the organelle's function are often lethal. Yet, relative to other organelles, little is known about how cells maintain and propagate peroxisomes or how they direct specific sets of newly synthesized proteins to these ...
H R, Waterham, J M, Cregg
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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.
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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.
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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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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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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.
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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.
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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, lipid metabolism, and peroxisomal disorders
Molecular Genetics and Metabolism, 2004Peroxisomes catalyse a large variety of different cellular functions of which most have to do with lipid metabolism. This paper deals with the role of peroxisomes in three key pathways of lipid metabolism, including: (1) etherphospholipid biosynthesis, (2) fatty acid beta-oxidation, and (3) fatty acid alpha-oxidation.
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Peroxisomal disorders can be classified as single-enzyme deficiencies or peroxisomal biogenesis disorders (characterized by multiple peroxisomal enzyme deficiencies or complete absence of peroxisomes). Most peroxisomal disorders give rise to complex multisystem disorders. Peroxisomal disorders associated with leukodystrophy are discussed in more detail,
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