HOMEPRODUCTSSERVICESCOMPANYCONTACTFAQResearchDictionaryPharmaMobileSign Up FREE or Login

pristanic acid

Also Known As:
2,6,10, 14-tetramethylpentadecanoic acid
Networked: 34 relevant articles (0 outcomes, 2 trials/studies)

Bio-Agent Context: Research Results

Experts

1. Wajner, Moacir: 3 articles (01/2014 - 03/2011)
2. Zanatta, Angela: 2 articles (01/2014 - 03/2011)
3. Viegas, Carolina Maso: 2 articles (01/2014 - 06/2011)
4. Busanello, Estela Natacha Brandt: 2 articles (01/2014 - 06/2011)
5. Kruska, Nicol: 2 articles (08/2011 - 11/2009)
6. Reiser, Georg: 2 articles (08/2011 - 11/2009)
7. Atshaves, Barbara P: 2 articles (02/2009 - 03/2005)
8. Payne, H Ross: 2 articles (02/2009 - 03/2005)
9. Kier, Ann B: 2 articles (02/2009 - 03/2005)
10. Schroeder, Friedhelm: 2 articles (02/2009 - 03/2005)

Related Diseases

1. Peroxisomal Disorders (Peroxisomal Disorder)
2. Refsum Disease (Refsum's Disease)
01/06/2009 - "While the clinical picture is reminiscent of Refsum disease, affected individuals have normal phytanic and pristanic acid levels in plasma, as well as normal enzymatic activity for alpha-oxidation. "
11/01/1992 - "Phytanic acid accumulated in plasma from the Refsum's disease patient [649 mumol/L, controls > 1 y (n = 100): < 10 mumol/L], whereas the pristanic acid concentration was within the control range [1.4 mumol/L, controls > 1 y (n = 100): < 3 mumol/L]. "
02/01/1988 - "As pristanic acid is present in normal or near-normal amounts in classical Refsum disease and rhizomelic chondrodysplasia, two disorders characterised by deficiencies in phytanic acid oxidation, we speculate that its accumulation is not secondary to a defect in the alpha-oxidation of phytanic acid, but is indicative of a block in the peroxisomal beta-oxidation of pristanic acid. "
05/01/1986 - "Patients with Refsum's disease lack the ability to degrade phytanic acid to pristanic acid and CO2. "
10/01/1992 - "Four different groups of diseases were characterized with a defective phytanic acid alpha-oxidation and/or pristanic acid beta-oxidation: 1) Refsum's disease, with a defect at phytanic acid alpha-hydroxylation; 2) rhizomelic chondrodysplasia punctata, with a defect at 2-hydroxyphytanic acid decarboxylation; 3) generalized peroxisomal disorders, with defects at 2-hydroxyphytanic acid decarboxylation and at pristanic acid beta-oxidation; 4) single peroxisomal beta-oxidation enzyme deficiencies, with a defect at pristanic acid beta-oxidation, resulting in an impaired phytanic acid alpha-oxidation by inhibition. "
3. Zellweger Syndrome (Zellweger's Syndrome)
4. Rhizomelic Chondrodysplasia Punctata
05/01/1996 - "In fibroblasts from a child with rhizomelic chondrodysplasia punctata the rate of degradation of U-3H- and 1-14C-labelled phytanic acid was markedly reduced whereas the rate of degradation of U-3H-labelled pristanic acid was normal. "
09/01/1993 - "In the blood spot from a patient with rhizomelic chondrodysplasia punctata, the concentration of phytanic acid was increased, whereas pristanic acid was within the control range, resulting in a low pristanic acid/phytanic acid ratio. "
10/01/1992 - "Four different groups of diseases were characterized with a defective phytanic acid alpha-oxidation and/or pristanic acid beta-oxidation: 1) Refsum's disease, with a defect at phytanic acid alpha-hydroxylation; 2) rhizomelic chondrodysplasia punctata, with a defect at 2-hydroxyphytanic acid decarboxylation; 3) generalized peroxisomal disorders, with defects at 2-hydroxyphytanic acid decarboxylation and at pristanic acid beta-oxidation; 4) single peroxisomal beta-oxidation enzyme deficiencies, with a defect at pristanic acid beta-oxidation, resulting in an impaired phytanic acid alpha-oxidation by inhibition. "
10/01/1998 - "Also in rhizomelic chondrodysplasia punctata, phytanic acid accumulates, owing to a deficiency in the peroxisomal import of proteins with a peroxisomal targeting sequence type 2. In patients affected with generalized peroxisomal disorders, degradation of both phytanic acid and pristanic acid is impaired owing to absence of functional peroxisomes. "
10/24/1997 - "In cells from patients with a defective alpha-oxidation (Refsum disease, rhizomelic chondrodysplasia punctata and generalized peroxisomal disorders) 2-hydroxyphytanic acid and pristanic acid were low or not detectable, showing that in these disorders the hydroxylation of phytanoyl-CoA to 2-hydroxyphytanoyl-CoA is deficient. "
5. Infantile Refsum Disease (Infantile Phytanic Acid Storage Disease)

Related Drugs and Biologics

1. Phytanic Acid
2. Fatty Acids (Saturated Fatty Acids)
3. Coenzyme A (CoA)
4. Bile Acids and Salts (Bile Acids)
5. Phytol
6. 2-hydroxyphytanic acid
7. Electron-Transferring Flavoproteins (Electron Transfer Flavoprotein)
8. Neurotransmitter Agents (Neurotransmitter)
9. Biological Markers (Surrogate Marker)
10. Vitamin E