Effects of phytanic acid on the vitamin E status, lipid composition and physical properties of retinal cell membranes: implications for adult Refsum disease

2001 ◽  
Vol 101 (6) ◽  
pp. 697 ◽  
Author(s):  
S. P. YOUNG ◽  
A. W. JOHNSON ◽  
D. P. R. MULLER
Keyword(s):  
Vitamin E ◽  
Physical Properties ◽  
Lipid Composition ◽  
Phytanic Acid ◽  
Cell Membranes ◽  
Retinal Cell ◽  
Refsum Disease ◽  
Vitamin E Status

Effects of phytanic acid on the vitamin E status, lipid composition and physical properties of retinal cell membranes: implications for adult Refsum disease

2001 ◽  
Vol 101 (6) ◽  
pp. 697-705 ◽  
Author(s):  
S.P. YOUNG ◽  
A.W. JOHNSON ◽  
D.P.R. MULLER
Keyword(s):  
Vitamin E ◽  
Phytanic Acid ◽  
Retinal Cell ◽  
Physical Parameters ◽  
Refsum Disease ◽  
Membrane Composition ◽  
Complex Situation ◽  
Retinal Cells ◽  
Membrane Function

Adult Refsum disease is an inherited disorder in which phytanic acid accumulates in tissues and serum. Two hypotheses have been proposed to explain the pathogenesis of this condition. The molecular distortion hypothesis suggests that phytanic acid may alter membrane composition and structure, thereby affecting membrane function(s). The anti-metabolite hypothesis suggests that an accumulation of phytanic acid in membranes may interfere with vitamin E function. These two hypotheses were investigated by studying the effects of modulating phytanic acid and α-tocopherol concentrations on the fatty acid composition and certain physical parameters of cultured retinal cells. Results showed that (a) the phospholipid fraction of retinal cells readily incorporated phytanic acid, (b) the incorporation of phytanic acid increased membrane fluidity, (c) there was no competition for uptake between phytanic acid and α-tocopherol, and (d) the incorporation of phytanic acid did not increase the susceptibility of membranes to lipid peroxidation in vitro. These results obtained with cultured retinal cells suggest that the molecular distortion hypothesis, but not the anti-metabolite hypothesis, could explain the pathogenesis of adult Refsum disease. In vitro tissue culture models can, however, only approximate to the much more complex situation that occurs in vivo.

scholarly journals Breeder Diet Strategies for Generating Ttpa-Null and Wild-Type Mice with Low Vitamin E Status to Assess Neurological Outcomes

2020 ◽  
Vol 4 (11) ◽  
Author(s):  
Katherine M Ranard ◽  
Matthew J Kuchan ◽  
John W Erdman
Keyword(s):  
Animal Model ◽  
Vitamin E ◽  
Mouse Model ◽  
Wild Type ◽  
Future Studies ◽  
Neurological Outcomes ◽  
Transfer Protein ◽  
And Function ◽  
The Brain ◽  
Vitamin E Status

ABSTRACT Studying vitamin E [α-tocopherol (α-T)] metabolism and function in the brain and other tissues requires an animal model with low α-T status, such as the transgenic α-T transfer protein (Ttpa)–null (Ttpa−/−) mouse model. Ttpa+/− dams can be used to produce Ttpa−/− and Ttpa+/+mice for these studies. However, the α-T content in Ttpa+/− dams’ diet requires optimization; diets must provide sufficient α-T for reproduction, while minimizing the transfer of α-T to the offspring destined for future studies that require low baseline α-T status. The goal of this work was to assess the effectiveness and feasibility of 2 breeding diet strategies on reproduction outcomes and offspring brain α-T concentrations. These findings will help standardize the breeding methodology used to generate the Ttpa−/− mice for neurological studies.

scholarly journals Selenium and Vitamin E Status: Impact on Viral Pathogenicity

2007 ◽  
Vol 137 (5) ◽  
pp. 1338-1340 ◽  
Author(s):  
Melinda A. Beck
Keyword(s):  
Vitamin E ◽  
Vitamin E Status

Oxidation resistance of LDL is correlated with vitamin E status in β-thalassemia intermedia

1998 ◽  
Vol 137 (2) ◽  
pp. 429-435 ◽  
Author(s):  
Luisa Tesoriere ◽  
Daniele D'Arpa ◽  
Aurelio Maggio ◽  
Vita Giaccone ◽  
Elisa Pedone ◽  
...  
Keyword(s):  
Vitamin E ◽  
Oxidation Resistance ◽  
Thalassemia Intermedia ◽  
Vitamin E Status
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