Article
Mutant fibrinogen cleared from the endoplasmic reticulum via endoplasmic reticulum-associated protein degradation and autophagy: an explanation for liver disease.
The American journal of pathology - 1 Apr 2006
Kruse Kristina B, Dear Amy, Kaltenbrun Erin R, Crum Brandan E, George Peter M, Brennan Stephen O, McCracken Ardythe A
Abstract excerpt
The endoplasmic reticulum (ER) quality control processes recognize and remove aberrant proteins from the secretory pathway. Several variants of the plasma protein fibrinogen are recognized as aberrant and degraded by ER-associated protein degradation (ERAD), thus leading to hypofibrinogenemia. A subset of patients with hypofibrinogenemia exhibit hepatic ER accumulation of the variant fibrinogens and develop liver...
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