Peroxisomal disorders.
The different types of adrenoleukodystrophy are considered with their clinical and biochemical features, particularly the excess of very-long-chain fatty acids. Then other conditions which show this latter finding are described, including the Zellweger cerebrohepatorenal syndrome, hyperpipecolic acidemia and Refsum disease. The role of peroxisomes is discussed and the different ways in which their functions can be disordered. The possibilities of treating these diseases is at the moment limited but examples are given of research already carried out in this field.
Gordon
N
N
Booth Hall Children's Hospital, Blackley, Manchester, England.
D000326:Adrenoleukodystrophy / Q000378:metabolism*; D001927:Brain Diseases / Q000378:metabolism*; D002549:Diffuse Cerebral Sclerosis of Schilder / Q000378:metabolism*; D005227:Fatty Acids / Q000378:metabolism*; D005260:Female; D006801:Humans; D008297:Male; D008830:Microbodies / Q000378:metabolism*; D013577:Syndrome
D005227:Fatty Acids