{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,8]],"date-time":"2025-10-08T22:30:21Z","timestamp":1759962621406},"reference-count":47,"publisher":"Wiley","issue":"4","license":[{"start":{"date-parts":[[1987,11,17]],"date-time":"1987-11-17T00:00:00Z","timestamp":564105600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J of Inher Metab Disea"],"published-print":{"date-parts":[[1988,12]]},"abstract":"<jats:title>Summary<\/jats:title><jats:p>Livers from seven patients with peroxisome disorders, three with Zellweger syndrome, one with infantile Refsum's syndrome and three with X\u2010linked adrenoleukodystrophy, were analysed by immunoblotting. The bifunctional protein catalysing two peroxisomal \u03b2\u2010oxidation reactions was deficient in all Zellweger livers and in the infantile Refsum's liver, consistent with the absence of morphologically recognizable peroxisomes. Three peroxisomal integral membrane proteins (IMPs) (69, 53 and 22 kDa) were present in normal amounts in all the Zellweger and adrenoleukodystrophy samples and they sedimented in a membrane fraction. These membrane proteins were also present in the infantile Refsum's liver. We suggest, on the basis of these results, that aberrant peroxisomal membranes may be present in Zellweger syndrome and that the defect is in the transport of matrix proteins into the organelle.<\/jats:p>","DOI":"10.1007\/bf01800425","type":"journal-article","created":{"date-parts":[[2005,6,21]],"date-time":"2005-06-21T17:01:24Z","timestamp":1119373284000},"page":"358-371","source":"Crossref","is-referenced-by-count":28,"title":["Peroxisomal integral membrane proteins in livers of patients with Zellweger syndrome, infantile Refsum's disease and X\u2010linked adrenoleukodystrophy"],"prefix":"10.1002","volume":"11","author":[{"given":"G. M.","family":"Small","sequence":"first","affiliation":[{"name":"The Rockefeller University  1230 York Avenue New York NY 10021 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"M. 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Kennedy Institute and the Departments of Neurology and Pediatrics Johns Hopkins University  Baltimore MD 21205 USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"P. 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