Recovery of PEX1-Gly843Asp associated peroxisome dysfunction by flavonoid compounds in fibroblasts from Zellweger spectrum patients
Zellweger spectrum disorder (ZSD) is due to defects in any one of 13 PEX proteins, encoded by PEX genes, which are required for peroxisome biogenesis. ZSD features neurologic, hepatic and renal abnormalities; however, one common mutation, PEX1-p.Gly843Asp (G843D), confers a milder phenotype and repr...
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| Format: | Others |
| Language: | en |
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McGill University
2013
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| Online Access: | http://digitool.Library.McGill.CA:80/R/?func=dbin-jump-full&object_id=114521 |