scholarly journals Slc25a17 Gene Trapped Mice: PMP34 Plays a Role in the Peroxisomal Degradation of Phytanic and Pristanic Acid

2020 ◽  
Vol 8 ◽  
Author(s):  
Paul P. Van Veldhoven ◽  
Evelyn de Schryver ◽  
Stephen G. Young ◽  
An Zwijsen ◽  
Marc Fransen ◽  
...  
Keyword(s):  
Pristanic Acid

Pristanic acid does not accumulate in peroxisomal acyl-CoA oxidase deficiency: Evidence for a Distinct peroxisomal pristanyl-CoA oxidase

1991 ◽  
Vol 14 (5) ◽  
pp. 681-684 ◽  
Author(s):  
H. J. ten Brink ◽  
B. T. Poll-The ◽  
J. M. Saudubray ◽  
R. J. A. Wanders ◽  
C. Jakobs
Keyword(s):  
Pristanic Acid ◽  
Oxidase Deficiency ◽  
Acyl Coa Oxidase

Pristanic Acid Provokes Lipid, Protein, and DNA Oxidative Damage and Reduces the Antioxidant Defenses in Cerebellum of Young Rats

2014 ◽  
Vol 13 (6) ◽  
pp. 751-759 ◽  
Author(s):  
Estela Natacha Brandt Busanello ◽  
Vannessa Gonçalves Araujo Lobato ◽  
Ângela Zanatta ◽  
Clarissa Günther Borges ◽  
Anelise Miotti Tonin ◽  
...  
Keyword(s):  
Oxidative Damage ◽  
Antioxidant Defenses ◽  
Pristanic Acid ◽  
Dna Oxidative Damage ◽  
Young Rats

Phytanic and pristanic acid content in Dutch farm milk and implications for the verification of the farming management system

2014 ◽  
Vol 35 (1) ◽  
pp. 21-24 ◽  
Author(s):  
Edoardo Capuano ◽  
Anjo Elgersma ◽  
Alba Tres ◽  
Saskia M. van Ruth
Keyword(s):  
Acid Content ◽  
Management System ◽  
Pristanic Acid

scholarly journals Formation of 2,3-pristenic acid and 3-hydroxypristanic acid from pristanic acid in human liver

1997 ◽  
Vol 20 (3) ◽  
pp. 441-443
Author(s):  
N. M. Verhoeven ◽  
D. S. M. Schor ◽  
G. A. Jansen ◽  
R. M. Kok ◽  
H. J. ten Brink ◽  
...  
Keyword(s):  
Human Liver ◽  
Pristanic Acid

Diagnosis of peroxisomal disorders by analysis of phytanic and pristanic acids in stored blood spots collected at neonatal screening

1993 ◽  
Vol 39 (9) ◽  
pp. 1904-1906 ◽  
Author(s):  
H J ten Brink ◽  
C M van den Heuvel ◽  
E Christensen ◽  
C Largillière ◽  
C Jakobs
Keyword(s):  
Neonatal Screening ◽  
Phytanic Acid ◽  
Zellweger Syndrome ◽  
Dried Blood Spots ◽  
Chondrodysplasia Punctata ◽  
Pristanic Acid ◽  
Rhizomelic Chondrodysplasia Punctata ◽  
Peroxisomal Disorders ◽  
Blood Spots ◽  
Blood Spot

Abstract Concentrations of phytanic acid and pristanic acid were measured in stored dried blood spots collected at neonatal screening from patients with peroxisomal disorders, and compared with concentrations in control blood spots. In blood spots from two patients with Zellweger syndrome both phytanic acid and pristanic acid concentrations were increased but their concentration ratio was normal. 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. In the blood spot from a patient with X-linked adrenoleukodystrophy, the concentrations of the acids and their ratio were normal. These findings are consistent with results for these acids in plasma from such patients. Measurement of phytanic acid and pristanic acid and their ratios in stored dried blood collected at neonatal screening can therefore be used in the diagnosis of peroxisomal disorders, especially for those cases in which, owing to early death of the patient, no other material is available for biochemical investigations.

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