Use of sodium 4-phenylbutyrate to define therapeutic parameters for reducing intracerebral hemorrhage and myopathy in Col4a1 mutant mice

Use of sodium 4-phenylbutyrate to define therapeutic parameters for reducing intracerebral hemorrhage and myopathy in Col4a1 mutant mice

Collagen type IV alpha 1 (COL4A1) and alpha 2 (COL4A2) form heterotrimers that constitute a major component of nearly all basement membranes. COL4A1 and COL4A2 mutations cause a multisystem disorder that includes variable cerebrovascular and skeletal muscle manifestations. The pathogenicity of COL4A...

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Bibliographic Details
Main Authors: Genki Hayashi, Cassandre Labelle-Dumais, Douglas B. Gould
Format: Article
Language:English
Published: The Company of Biologists 2018-07-01
Series:Disease Models & Mechanisms
Subjects:
Cerebral small vessel disease
Stroke
Intracerebral hemorrhage
Collagen
COL4A1
Myopathy
Drug therapy
Chaperones
Online Access:http://dmm.biologists.org/content/11/7/dmm034157

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