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Pathogenesis of peroxisomal deficiency disorders (Zellweger syndrome) may be mediated by misregulation of the GABAergic system via the diazepam binding inhibitor
BACKGROUND: Zellweger syndrome (ZS) is a fatal inherited disease caused by peroxisome biogenesis deficiency. Patients are characterized by multiple disturbances of lipid metabolism, profound hypotonia and neonatal seizures, and distinct craniofacial malformations. Median live expectancy of ZS patien...
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| Format: | Artigo |
| Sprache: | Inglês |
| Veröffentlicht: |
BioMed Central
2004
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| Online Zugang: | https://ncbi.nlm.nih.gov/pmc/articles/PMC391370/ https://ncbi.nlm.nih.gov/pubmed/15102341 https://ncbi.nlm.nih.govhttp://dx.doi.org/10.1186/1471-2431-4-5 |
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