title: Zellweger-like phenotype in two siblings: a defect in peroxisomal beta-oxidation with elevated very long-chain fatty acids but normal bile acids.
authors.affiliation: Department of Pediatrics, Rambam Medical Center, Haifa, Israel.
authors.forename: H
authors.initials: H
authors.lastname: Mandel
authors.forename: M
authors.initials: M
authors.lastname: Berant
authors.forename: A
authors.initials: A
authors.lastname: Aizin
authors.forename: R
authors.initials: R
authors.lastname: Gershony
authors.forename: S
authors.initials: S
authors.lastname: Hemmli
authors.forename: R B
authors.initials: RB
authors.lastname: Schutgens
authors.forename: R J
authors.initials: RJ
authors.lastname: Wanders
mesh_terms: D001647:Bile Acids and Salts / Q000378:metabolism*; D005227:Fatty Acids / Q000097:blood / Q000378:metabolism*; D005260:Female; D005347:Fibroblasts / Q000378:metabolism; D006801:Humans; D015151:Immunoblotting; D007231:Infant, Newborn; D008099:Liver / Q000648:ultrastructure; D008297:Male; D008830:Microbodies / Q000378:metabolism*; D010084:Oxidation-Reduction; D010641:Phenotype; D015211:Zellweger Syndrome / Q000378:metabolism*
chemical_list: D001647:Bile Acids and Salts; D005227:Fatty Acids