Chemical chaperone treatment reduces intracellular accumulation of mutant collagen IV and ameliorates the cellular phenotype of a COL4A2 mutation that causes haemorrhagic stroke. [electronic resource]
Producer: 20140805Description: 283-92 p. digitalISSN:- 1460-2083
- Apoptosis -- drug effects
- Basement Membrane -- drug effects
- Brain Diseases -- drug therapy
- Cell Proliferation -- drug effects
- Cells, Cultured
- Collagen Type IV -- deficiency
- Endoplasmic Reticulum Stress -- drug effects
- Female
- Hemiplegia -- drug therapy
- Humans
- Intracranial Hemorrhages -- complications
- Male
- Mutation
- Phenotype
- Phenylbutyrates -- pharmacology
- Porencephaly
- Proteasome Endopeptidase Complex -- metabolism
- Stroke -- complications
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Publication Type: Journal Article; Research Support, Non-U.S. Gov't
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