We created a Ptpn11 LOH mouse model in PRRX1-expressing cells, and found that SHP2 deficiency in PRRX1+ OCPs was involved in the pathogenesis of cartilage lesions, indicating that SHP2 functions as a tumor suppressor in cartilage and is required in OCPs for cartilage development and homeostasis. Here, PRRX1 is linked to neoplasm.