Nonsense suppressor therapies rescue peroxisome lipid metabolism and assembly in cells from patients with specific PEX gene mutations. [electronic resource]
- Journal of cellular biochemistry May 2011
- 1250-8 p. digital
Publication Type: Journal Article; Research Support, N.I.H., Extramural
1097-4644
10.1002/jcb.22979 doi
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