Article
Altered quality control in the endoplasmic reticulum causes cortical dysplasia in knock-in mice expressing a mutant BiP.
Molecular and cellular biology - 1 Jan 2008
Mimura Naoya, Yuasa Shigeki, Soma Miho, Jin Hisayo, Kimura Keita, Goto Shigemasa, Koseki Haruhiko, Aoe Tomohiko
Abstract excerpt
Binding immunoglobulin protein (BiP) is an endoplasmic reticulum (ER) molecular chaperone that is central to ER function. We examined knock-in mice expressing a mutant BiP in order to elucidate physiological processes that are sensitive to BiP functions during development and adulthood. The mutant BiP lacked the retrieval sequence that normally functions to return BiP to the ER from the secretory pathway. This...
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