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1.
Sci Rep ; 7(1): 12699, 2017 10 05.
Article in English | MEDLINE | ID: mdl-28983104

ABSTRACT

Transdifferentiation of hypertrophic chondrocytes into bone-forming osteoblasts has been reported, yet the underlying molecular mechanism remains incompletely understood. SHP2 is an ubiquitously expressed cytoplasmic protein tyrosine phosphatase. SHP2 loss-of-function mutations in chondroid cells are linked to metachondromatosis in humans and mice, suggesting a crucial role for SHP2 in the skeleton. However, the specific role of SHP2 in skeletal cells has not been elucidated. To approach this question, we ablated SHP2 in collagen 2α1(Col2α1)-Cre- and collagen 10α1(Col10α1)-Cre-expressing cells, predominantly proliferating and hypertrophic chondrocytes, using "Cre-loxP"-mediated gene excision. Mice lacking SHP2 in Col2α1-Cre-expressing cells die at mid-gestation. Postnatal SHP2 ablation in the same cell population caused dwarfism, chondrodysplasia and exostoses. In contrast, mice in which SHP2 was ablated in the Col10α1-Cre-expressing cells appeared normal but were osteopenic. Further mechanistic studies revealed that SHP2 exerted its influence partly by regulating the abundance of SOX9 in chondrocytes. Elevated and sustained SOX9 in SHP2-deficient hypertrophic chondrocytes impaired their differentiation to osteoblasts and impaired endochondral ossification. Our study uncovered an important role of SHP2 in bone development and cartilage homeostasis by influencing the osteogenic differentiation of hypertrophic chondrocytes and provided insight into the pathogenesis and potential treatment of skeletal diseases, such as osteopenia and osteoporosis.


Subject(s)
Bone Neoplasms/genetics , Chondromatosis/genetics , Exostoses, Multiple Hereditary/genetics , Osteogenesis/genetics , Protein Tyrosine Phosphatase, Non-Receptor Type 11/genetics , SOX9 Transcription Factor/genetics , Animals , Bone Development/genetics , Bone Neoplasms/physiopathology , Cartilage/growth & development , Cartilage/metabolism , Cartilage/pathology , Cell Differentiation/genetics , Cell Proliferation/genetics , Cell Transdifferentiation/genetics , Chondrocytes/metabolism , Chondrocytes/pathology , Chondrogenesis/genetics , Chondromatosis/physiopathology , Exostoses, Multiple Hereditary/physiopathology , Growth Plate/growth & development , Growth Plate/metabolism , Growth Plate/pathology , Humans , Hypertrophy/genetics , Hypertrophy/pathology , Mice , Osteoblasts/metabolism
2.
J Voice ; 27(2): 255-7, 2013 Mar.
Article in English | MEDLINE | ID: mdl-23406842

ABSTRACT

Laryngeal chondromas are uncommon, benign, slow-growing neoplasms with few reports in the literature. Vocal fold chondromas are even more rare, and all reported cases are unilateral. Here, we present the first case of bilateral vocal fold chondromas. Detailed evaluation, careful resection with phonomicrosurgery technique, and perioperative voice therapy are considered essential for the management.


Subject(s)
Chondromatosis , Laryngeal Neoplasms , Vocal Cords , Chondromatosis/pathology , Chondromatosis/physiopathology , Chondromatosis/surgery , Humans , Laryngeal Neoplasms/pathology , Laryngeal Neoplasms/physiopathology , Laryngeal Neoplasms/surgery , Laryngoscopy/methods , Male , Microsurgery , Middle Aged , Phonation , Predictive Value of Tests , Plastic Surgery Procedures , Recovery of Function , Treatment Outcome , Vocal Cords/pathology , Vocal Cords/physiopathology , Vocal Cords/surgery , Voice Quality , Voice Training
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