Peroxisome biogenesis disorders: The role of peroxisomes and metabolic dysfunction in developing brain
โ Scribed by P. L. Faust; D. Banka; R. Siriratsivawong; V. G. Ng; T. M. Wikander
- Publisher
- Springer
- Year
- 2005
- Tongue
- English
- Weight
- 754 KB
- Volume
- 28
- Category
- Article
- ISSN
- 0141-8955
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The PEX6 (peroxisome assembly factor-2, PAF-2) gene encodes a member of the AAA protein (ATPases associated with diverse cellular activities) family and restores peroxisome assembly in fibroblasts from peroxisome biogenesis disorder patients belonging to complementation group C (group 4 in the Unite
Thioridazine, an inhibitor of peroxisomal beta-oxidation, was administered orally to nursing rats during the period of maximal myelination in the pups (8-21 days postnatally). Under the experimental conditions, thioridazine causes accumulation of C24 and C26 fatty acids in pup brain lipids, an effec