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

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Hayashi, Genki ; Labelle-Dumais, Cassandre ; Gould, Douglas B. (2018)
  • Publisher: The Company of Biologists Ltd
  • Journal: Disease Models & Mechanisms, volume 11, issue 7 (issn: 1754-8403, eissn: 1754-8411)
  • Related identifiers: doi: 10.1242/dmm.034157, pmc: PMC6078406
  • Subject: RB1-214 | Drug therapy | Pathology | Research Article | Myopathy | Collagen | Cerebral small vessel disease | Medicine | Stroke | Intracerebral hemorrhage | R | COL4A1 | Chaperones

ABSTRACT 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 man... View more