Urinary polyamine and metabolite excretion by children with Zellweger's syndrome.
In order to investigate whether, due to a lack of peroxisomes, polyamine degradation is altered in patients with the cerebro-hepato-renal syndrome of Zellweger, we determined total, free and acetylated polyamines and some of their catabolites in urines of six patients and age-matched healthy children. The normal polyamine excretion patterns of the patients, compared to the control group, suggest that either the intracellular localisation of the polyamine degrading enzyme, polyamine oxidase, is not exclusively limited to peroxisomes or that the enzyme is located in the peroxisomal matrix.
Govaerts
L C
LC
Department of Human Genetics, Catholic University of Nijmegen, St. Radboud Hospital, The Netherlands.
van den Berg
G A
GA
Theeuwes
A
A
Muskiet
F A
FA
Monnens
L A
LA
D000599:Amino Acids, Diamino / Q000652:urine; D002675:Child, Preschool; D005260:Female; D006801:Humans; D006868:Hydrolysis; D007223:Infant; D007231:Infant, Newborn; D011073:Polyamines / Q000378:metabolism / Q000652:urine*; D013095:Spermidine / Q000031:analogs & derivatives / Q000652:urine; D013096:Spermine / Q000031:analogs & derivatives / Q000652:urine; D015211:Zellweger Syndrome / Q000378:metabolism / Q000652:urine*
D000599:Amino Acids, Diamino; D011073:Polyamines; C017988:N(1)-acetylspermidine; C029452:N'-acetylspermine; D013096:Spermine; C035160:isoputreanine; C029453:N',N''-diacetylspermine; D013095:Spermidine