MRI evidence that docosahexaenoic acid ethyl ester improves myelination in generalized peroxisomal disorders

@article{Martnez1998MRIET,
  title={MRI evidence that docosahexaenoic acid ethyl ester improves myelination in generalized peroxisomal disorders},
  author={Manuela Mart{\'i}nez and {\'E}lida V{\'a}zquez},
  journal={Neurology},
  year={1998},
  volume={51},
  pages={26 - 32}
}
Objective: We have found that docosahexaenoic acid (DHA; 22:6n-3), an important constituent of the CNS and retina, is dramatically decreased in patients with generalized peroxisomal disorders. Such a DHA deficiency could be pathogenic. Our aim was to test the possible beneficial effects of normalizing the DHA levels in these patients. The current report focuses on MRI changes during the treatment and reports follow-up evidence of myelin improvement in five peroxisomal disorder patients treated… 

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References

SHOWING 1-10 OF 26 REFERENCES
Docosahexaenoic acid–A new therapeutic approach to peroxisomal‐disorder patients
TLDR
The present data suggest that DHA deficiency may be the cause for some of the most characteristic abnormalities in peroxisomal-disorder patients and open new therapeutic possibilities for these patients.
Severe deficiency of docosahexaenoic acid in peroxisomal disorders
TLDR
The findings reported here strongly reinforce the hypothesis of a new enzymatic defect in peroxisomal disorders involving the desaturation of long polyunsaturated fatty acids, especially of the ω3 family.
Docosahexaenoic acid synthesis in human skin fibroblasts involves peroxisomal retroconversion of tetracosahexaenoic acid.
TLDR
The results demonstrate that the pathway for docosahexaenoic acid synthesis in human cells involves 24-carbon intermediates, which may underlie some of the pathology that occurs in genetic diseases involving peroxisomal beta-oxidation.
Lignoceric acid is oxidized in the peroxisome: implications for the Zellweger cerebro-hepato-renal syndrome and adrenoleukodystrophy.
TLDR
It is found that normal hepatocellular peroxisomes are found in the liver biopsy of a childhood ALD patient and the oxidation of palmitic acid in cultured skin fibroblasts was inhibited by 62% in control and X chromosome-linked ALD patients compared with 88% in CHRS and neonatal ALD.
Defects of bile acid synthesis in Zellweger's syndrome.
TLDR
Patients with Zellweger's syndrome were examined for the presence of mitochondrial defects in bile acid synthesis and these findings give added support to the role of mitochondrial oxidative side chain cleavage in the overall scheme of bile acids synthesis.
Biochemical and functional effects of prenatal and postnatal omega 3 fatty acid deficiency on retina and brain in rhesus monkeys.
TLDR
It is suggested that dietary omega 3 fatty acids are retina and brain, and abnormally low levels of 22:6 omega 3 may produce alterations in the biophysical properties of photoreceptor and neural membranes that may underlie these functional impairments.
Severe plasmalogen deficiency in tissues of infants without peroxisomes (Zellweger syndrome)
The Zellweger syndrome is a lethal hereditary disease characterized by the absence of peroxisomes (microbodies) in liver and kidney, and variable abnormalities in mitochondria1–8. We show here that
Cerebro-hepato-renal (Zellweger) syndrome, adrenoleukodystrophy, and Refsum's disease: Plasma changes and skin fibroblast phytanic acid oxidase
TLDR
The great similarity observed in neonatal adrenoleukodystrophy/Zellweger syndrome and infantile Refsum's disease suggests that the basic biochemical lesion in each may be similar or at least closely related.
Neonatal adrenoleukodystrophy: clinical, pathologic, and biochemical delineation of a syndrome affecting both males and females.
TLDR
The basic biochemical defect appears to be a diminished capacity to oxidize fatty acids leading to accumulation in cholesterol esters, which is most probably autosomally recessive in inheritance.
...
1
2
3
...