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

• Hoofdauteurs: Genki Hayashi (Auteur), Cassandre Labelle-Dumais (Auteur), Douglas B. Gould (Auteur)
• Formaat: Boek
• Gepubliceerd in: The Company of Biologists, 2018-07-01T00:00:00Z.
• Onderwerpen: article
• Online toegang: Connect to this object online.

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...