Distinct immuno-localization of mucin and other biliary proteins in human cholesterol gallstones

Autor: J.D. Ostrow, Nicole Domingo, P. Lechene de la Porte, Huguette Lafont, M.J.A. van Wijland, A. K. Groen

Publikováno v: Journal of hepatology, 25(3), 339-348. Elsevier

Background/Aims: Cholesterol gallstones consist of cholesterol crystals and smaller amounts of pigments and calcium salts, arrayed on a mucin plus protein matrix. The localization of the various biliary proteins in the stones has not been characteriz

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::c8efa2efbca5b36a530e42fae00892a2
https://pubmed.ncbi.nlm.nih.gov/8895014

Direct demonstration that the deficient oxidation of very long chain fatty acids in X-linked adrenoleukodystrophy is due to an impaired ability of peroxisomes to activate very long chain fatty acids

Autor: H. van den Bosch, M.J.A. van Wijland, Ruud B.H. Schutgens, C. W. T. van Roermund, AndréW. Schram, R. J. A. Wanders, Joseph M. Tager

Publikováno v: Biochemical and biophysical research communications, 153(2), 618-624. Academic Press Inc.

A method was developed to prepare peroxisome-enriched fractions depleted of microsomes and mitochondria from cultured skin fibroblasts. The method consists of differential centrifugation of a postnuclear supernatant followed by density gradient centr

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::6a3e0fafc7b23d73a70ca917931abd19
https://doi.org/10.1016/s0006-291x(88)81140-9

Peroxisomal fatty acid beta-oxidation in relation to the accumulation of very long chain fatty acids in cultured skin fibroblasts from patients with Zellweger syndrome and other peroxisomal disorders

Autor: H. van den Bosch, AndréW. Schram, R. J. A. Wanders, C. W. T. van Roermund, Judith C. Heikoop, Joseph M. Tager, M.J.A. van Wijland, Ruud B.H. Schutgens

Publikováno v: Journal of clinical investigation, 80(6), 1778-1783. The American Society for Clinical Investigation

The peroxisomal oxidation of the long chain fatty acid palmitate (C16:0) and the very long chain fatty acids lignocerate (C24:0) and cerotate (C26:0) was studied in freshly prepared homogenates of cultured skin fibroblasts from control individuals an

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::530f50d677b15fff8fea5ddc2cc11195
https://doi.org/10.1172/jci113271

The Peroxisomal β-Oxidation Systems: Characteristics and (Dys) Functions in Man

Autor: Ruud B.H. Schutgens, C. W. T. van Roermund, H. van den Bosch, R. J. A. Wanders, Joseph M. Tager, M.J.A. van Wijland

Publikováno v: Molecular Basis of Membrane-Associated Diseases ISBN: 9783642744174

Peroxisomes are now known to be present in virtually every mammalian cell except the mature erythrocyte. They were first described by Rhodin in 1954 as “spheric or oval bodies” present in the cytoplasm of mouse proximal kidney tubules [1]. Eviden

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_________::90148caf461492b11d236750375f7c07
https://doi.org/10.1007/978-3-642-74415-0_34

Prenatal diagnosis of Zellweger syndrome by measurement of very long chain fatty acid (C26 : 0) β-oxidation in cultured chorionic villous fibroblasts: Implications for early diagnosis of other peroxisomal disorders

Autor: A. Tromp, A. A. Nijenhuis, H. van den Bosch, Joseph M. Tager, R. J. A. Wanders, C. W. T. van Roermund, Ruud B.H. Schutgens, M.J.A. van Wijland

Publikováno v: Clinica Chimica Acta, 165(2-3), 303. Elsevier
Clinica chimica acta; international journal of clinical chemistry, 165(2-3), 303-310. Elsevier

In this paper we show that cultured chorionic villous fibroblasts efficiently catalyse the peroxisomal beta-oxidation of hexacosanoic acid (cerotic acid), a saturated very long chain fatty acid containing 26 carbon atoms. Hexacosanoic beta-oxidation

Externí odkaz: https://explore.openaire.eu/search/publication?articleId=doi_dedup___::fd9562499cc73bd68648616e925f1966
https://dspace.library.uu.nl/handle/1874/17265