Ontology highlight
ABSTRACT:
INSTRUMENT(S): LTQ Orbitrap Velos
ORGANISM(S): Mus Musculus (mouse)
TISSUE(S): Epiphyseal Growth Plate, Hypertrophic Chondrocyte
DISEASE(S): Schmid Metaphyseal Chondrodysplasia
SUBMITTER: MATEUSZ KUDELKO
LAB HEAD: Danny Chan
PROVIDER: PXD002125 | Pride | 2015-11-25
REPOSITORIES: Pride
Kudelko Mateusz M Chan Cecilia W L CW Sharma Rakesh R Yao Qing Q Lau Edward E Chu Ivan K IK Cheah Kathryn S E KS Tanner Julian A JA Chan Danny D
Journal of proteome research 20151211 1
Emerging evidence implicates ER stress caused by unfolded mutant proteins in chondrocytes as the underlying pathology of chondrodysplasias. ER stress is triggered in hypertrophic chondrocytes (HCs) in a mouse model (13del) of metaphyseal chondrodysplasia type Schmid (MCDS) caused by misfolded mutant collagen X proteins, but the HCs do not undergo apoptosis; rather chondrocyte differentiation is altered, causing skeletal abnormality. How 13del HCs can escape from apoptosis and survive ER stress i ...[more]