Matches in UGent Biblio for { <https://biblio.ugent.be/publication/887286#aggregation> ?p ?o. }
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- aggregation classification "A1".
- aggregation creator B511514.
- aggregation creator B511515.
- aggregation creator B511516.
- aggregation creator person.
- aggregation creator person.
- aggregation creator person.
- aggregation creator person.
- aggregation date "1991".
- aggregation hasFormat 887286.bibtex.
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- aggregation hasFormat 887286.doc.
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- aggregation isPartOf urn:issn:0174-7398.
- aggregation language "eng".
- aggregation subject "Biology and Life Sciences".
- aggregation title "Peroxisomal localization of the immunoreactive β-oxidation enzymes in a neonate with a β-oxidation defect: pathological observations in liver, adrenal cortex and kidney".
- aggregation abstract "A boy born to healthy, unrelated parents, presented at birth with hypotonia and seizures. Very long chain fatty acids in the plasma were strongly elevated; bile acid intermediates and plasmalogen biosynthesis were normal. Acyl-CoA oxidase activity was normal. The patient died at the age of 3 months. The cerebellum and medulla oblongata showed neuronal migration defects. The specific biochemical basis for the impaired peroxisomal beta-oxidation has not been found. The three immunoreactive peroxisomal beta-oxidation enzymes and catalase were localized in the hepatocellular peroxisomes. Aberrant features of the peroxisomes included: a subpopulation of organelles larger than 1-mu-m, an amorphous nucleoid in many organelles, and invaginations of the peroxisomal membrane into the matrix. Peroxisomes in the proximal renal tubules also contained the three immunoreactive beta-oxidation enzymes. Regularly spaced trilamellar inclusions were seen in hepatic macrophages; they were much more abundant in adrenocortical macrophages. The inclusions were birefringent and resistant to acetone extraction. Distinct hepatic fibrosis had developed over a period of 2.5 months. We speculate that the impaired beta-oxidation is due to a defect at the level of the peroxisomal carnitine octanoyl or -acetyl transferase, responsible for the export of beta-oxidation products.".
- aggregation authorList BK857764.
- aggregation endPage "308".
- aggregation issue "4".
- aggregation startPage "301".
- aggregation volume "419".
- aggregation isDescribedBy 887286.
- aggregation similarTo BF01606521.
- aggregation similarTo LU-887286.