Analyze Mouse Knockout Models of UPR Pathway Elements.

Evidence from genetic studies in human and mice indicates that defective skeletal development is one of the major phenotypic outcomes for aberrant UPR signaling. Visualization of morphological alterations in whole-mount skeleton and protein secretion and UPR activation on tissue sections is the very first step to investigate skeletal phenotypes of UPR-related mouse models. In this chapter, we introduce the major techniques that have been frequently used in our laboratory to study UPR-induced skeletal disorders with genetically modified mice and provide descriptive directions of mouse genotyping, bone tissue grossing, whole-mount skeletal staining, immunostaining assays of matrix secretion, and UPR activation.