Oxidation product of fatty acids in the diet, particularly phytanic acid. Phytanic acid is oxidized by alpha oxidation yielding pristanic acid, which is subsequently degraded by peroxisomal beta oxidation. Several inborn errors of metabolism with one or more deficiencies in the phytanic acid and pristanic acid breakdown have been described.
European journal of pediatrics, 153(7 Suppl 1), S44-S48 (1994-01-01)
Peroxisomal disorders represent a recently recognized group of inherited diseases in man, now comprising 14 different disorders. If discussion is restricted to those peroxisomal disorders in which there is neurological involvement (thereby excluding hyperoxaluria and acatalasaemia), results over the least
To identify prognostic markers reflecting the extent of peroxisome dysfunction in primary skin fibroblasts from patients with peroxisome biogenesis disorders (PBD). PBD are a genetically heterogeneous group of disorders due to defects in at least 11 distinct genes. Zellweger syndrome
Biochemical markers predicting survival in peroxisome biogenesis disorders.
Jeannette Gootjes et al.
Advances in experimental medicine and biology, 544, 67-68 (2004-01-10)
Cancer epidemiology, biomarkers & prevention : a publication of the American Association for Cancer Research, cosponsored by the American Society of Preventive Oncology, 12(8), 775-783 (2003-08-15)
An enzyme previously identified as alpha-methylacyl-CoA racemase (AMACR) is overexpressed in high-grade prostatic intraepithelial neoplasia and in a majority (60-100%) of prostate cancers (CaPs) as compared with normal and benign hyperplastic lesions of the prostate, where it is minimally expressed.
Phytanic and pristanic acid are naturally occuring ligands.
Anna W M Zomer et al.
Advances in experimental medicine and biology, 544, 247-254 (2004-01-10)
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