PEROXISOMAL ORGANIZATION IN NORMAL AND CEREBROHEPATORENAL (ZELLWEGER) SYNDROME FIBROBLASTS

Simple item page
dc.contributor.authorSANTOS, MJ
dc.contributor.authorOJEDA, JM
dc.contributor.authorGARRIDO, J
dc.contributor.authorLEIGHTON, F
dc.date.accessioned2025-01-23T19:27:39Z
dc.date.available2025-01-23T19:27:39Z
dc.date.issued1985
dc.description.abstractThe reported absence of morphologically detectable peroxisomes in liver and kidney tissue cells from patients affected by the autosomic recessive, inherited metabolic disease known as cerebrohepatorenal, or Zellweger, syndrome was studied in fibroblasts, assuming it to be a generalized defect. Normal cultured fibroblasts were shown to contain peroxisomes according to morphological, biochemical, and subcellular fractionation criteria: particle-bound catalase and fatty acyl-CoA oxidase copurify in subcellular fractionation by differential centrifugation or isopycnic equilibrium in continuous density gradients and peroxidase-positive organelles of .apprxeq. 0.1 .mu.m in diameter are detected in the cytoplasm. In Zellweger cultured fibroblasts, these peroxisomal enzymes are present; however, they behave as cytosolic enzymes in the different subcellular fractionation procedures employed and peroxisomes are not detected cytochemically. These findings support the hypothesis that the lack of peroxisomes in this genetic disease is the consequence of a defect in the assembly of the peroxisomal constituents. Furthermore, the value of fibroblasts for subcellular analysis of peroxisomal defects is illustrated.
dc.fuente.origenWOS
dc.identifier.eissn1091-6490
dc.identifier.issn0027-8424
dc.identifier.urihttps://repositorio.uc.cl/handle/11534/99751
dc.identifier.wosidWOS:A1985ARZ4800041
dc.issue.numero19
dc.language.isoen
dc.pagina.final6560
dc.pagina.inicio6556
dc.revistaProceedings of the national academy of sciences of the united states of america
dc.rightsacceso restringido
dc.subject.ods03 Good Health and Well-being
dc.subject.odspa03 Salud y bienestar
dc.titlePEROXISOMAL ORGANIZATION IN NORMAL AND CEREBROHEPATORENAL (ZELLWEGER) SYNDROME FIBROBLASTS
dc.typeartículo
dc.volumen82
sipa.indexWOS
sipa.trazabilidadWOS;2025-01-12
Files
Collections
Artículos de revistas