Article
Antioxidant and anti-inflammatory agents mitigate pathology in a mouse model of pseudoachondroplasia.
Human molecular genetics - 15 Jul 2015
Posey Karen L, Coustry Francoise, Veerisetty Alka C, Hossain Mohammad, Alcorn Joseph L, Hecht Jacqueline T
Abstract excerpt
Pseudoachondroplasia (PSACH), a severe short-limb dwarfing condition, results from mutations that cause misfolding of the cartilage oligomeric matrix protein (COMP). Accumulated COMP in growth plate chondrocytes activates endoplasmic reticulum stress, leading to inflammation and chondrocyte death. Using a MT-COMP mouse model of PSACH that recapitulates the molecular and clinical PSACH phenotype, we previously...
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