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Intestinal cell kinase regulates chondrocyte proliferation and maturation during skeletal development

Mengmeng Ding, Li Jin, Lin Xie, So Hyun Park, Yixin Tong, Di Wu, Zheng Fu, View ORCID ProfileXudong Li
doi: https://doi.org/10.1101/139089
Mengmeng Ding
1Department of Orthopaedic Surgery, University of Virginia, Charlottesville, VA 22908, USA
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Li Jin
1Department of Orthopaedic Surgery, University of Virginia, Charlottesville, VA 22908, USA
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Lin Xie
1Department of Orthopaedic Surgery, University of Virginia, Charlottesville, VA 22908, USA
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So Hyun Park
2Department of Pharmacology, University of Virginia, Charlottesville, Virginia 22908, USA
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Yixin Tong
2Department of Pharmacology, University of Virginia, Charlottesville, Virginia 22908, USA
3The Gastrointestinal Surgery Center, Tongji Hospital, Huazhong University of Science & Technology, Hubei, 430030, China
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Di Wu
2Department of Pharmacology, University of Virginia, Charlottesville, Virginia 22908, USA
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Zheng Fu
2Department of Pharmacology, University of Virginia, Charlottesville, Virginia 22908, USA
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  • For correspondence: xl2n@virginia.edu zf6n@virginia.edu
Xudong Li
1Department of Orthopaedic Surgery, University of Virginia, Charlottesville, VA 22908, USA
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  • ORCID record for Xudong Li
  • For correspondence: xl2n@virginia.edu zf6n@virginia.edu
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Abstract

An autosomal recessive loss-of-function mutation R272Q in human ICK (intestinal cell kinase) gene causes profound multiplex developmental defects in human ECO (endocrine-cerebro-osteodysplasia) syndrome. ECO patients exhibit a wide variety of skeletal abnormalities, yet the underlying cellular and molecular mechanisms by which ICK regulates skeletal development remain largely unknown. The goal of this study is to understand the structural and mechanistic basis underlying skeletal anomalies caused by ICK dysfunction. Ick R272Q knock in transgenic mouse model not only recapitulated major ECO skeletal defects such as short limbs and polydactyly but also revealed a deformed spine with deficient intervertebral disc. Loss of ICK functions markedly reduces mineralization in the spinal column, ribs, and long bones. Ick mutants show a significant decrease in the number of proliferating chondrocytes and type X collagen-expressing hypertrophic chondrocytes in the spinal column and the growth plate of long bones. Our results demonstrate that ICK plays an important role in bone and intervertebral disc development by promoting chondrocyte proliferation and maturation, and thus provide novel mechanistic insights into the skeletal phenotypes of human ECO syndrome.

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Posted May 17, 2017.
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Intestinal cell kinase regulates chondrocyte proliferation and maturation during skeletal development
Mengmeng Ding, Li Jin, Lin Xie, So Hyun Park, Yixin Tong, Di Wu, Zheng Fu, Xudong Li
bioRxiv 139089; doi: https://doi.org/10.1101/139089
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Intestinal cell kinase regulates chondrocyte proliferation and maturation during skeletal development
Mengmeng Ding, Li Jin, Lin Xie, So Hyun Park, Yixin Tong, Di Wu, Zheng Fu, Xudong Li
bioRxiv 139089; doi: https://doi.org/10.1101/139089

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