Nonsense suppressor therapies rescue peroxisome lipid metabolism and assembly in cells from patients with specific PEX gene mutations. [electronic resource]
Producer: 20110720Description: 1250-8 p. digitalISSN:- 1097-4644
- Alleles
- Animals
- CHO Cells
- Chondrodysplasia Punctata, Rhizomelic -- metabolism
- Codon, Nonsense -- drug effects
- Cricetinae
- Cricetulus
- Female
- Fibroblasts -- metabolism
- Gentamicins -- therapeutic use
- Humans
- Lipid Metabolism -- genetics
- Membrane Proteins -- genetics
- Oxadiazoles -- therapeutic use
- Peroxisomal Biogenesis Factor 2
- Peroxisomal Disorders -- drug therapy
- Peroxisomal Targeting Signal 2 Receptor
- Plasmalogens -- genetics
- Receptors, Cytoplasmic and Nuclear -- metabolism
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Publication Type: Journal Article; Research Support, N.I.H., Extramural
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