GdX/UBL4A null mice exhibit mild kyphosis and scoliosis accompanied by dysregulation of osteoblastogenesis and chondrogenesis

J. Liang, J. Li, Y. Fu, F. Ren, J. Xu, M. Zhou, P. Li, H. Feng, Y. Wang

Research output: Contribution to journalArticle

3 Citations (Scopus)
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Abstract

GdX, also named ubiquitin-like protein 4A, is a ubiquitin-domain protein characterized by a ubiquitin-like domain that regulates the movement of misfolded proteins from the endoplasmic reticulum membrane to proteasome. However, its function in skeletal biology remains unclear. Here, we report that GdX plays a crucial role in skeletal development as mice lacking GdX exhibit skeletal dysplasias, mild kyphosis, and scoliosis. During embryonic stage, GdX knockout mice display decreased bone mineral density and trabecular bone accompanied by delayed osteogenic formation. GdX knockout mice also have blended spine and small body size. At the molecular level, GdX knockout mice showed perturbed expression of osteogenesis-related genes and cartilage developmental genes, indicative of altered differentiation of mesenchymal cell lineage. Collectively, our results uncovered GdX as a novel regulator in bone development and a potential candidate gene for skeletal dysplasias.

Original languageEnglish
Pages (from-to)129-136
Number of pages8
JournalCell Biochemistry and Function
Volume36
Issue number3
DOIs
Publication statusPublished - 1 Apr 2018

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Chondrogenesis
Kyphosis
Scoliosis
Knockout Mice
Bone
Genes
Ubiquitin
Ubiquitins
Developmental Genes
Bone Development
Cartilage
Body Size
Cell Lineage
Proteasome Endopeptidase Complex
Osteogenesis
Endoplasmic Reticulum
Bone Density
Minerals
Proteins
Spine

Cite this

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title = "GdX/UBL4A null mice exhibit mild kyphosis and scoliosis accompanied by dysregulation of osteoblastogenesis and chondrogenesis",
abstract = "GdX, also named ubiquitin-like protein 4A, is a ubiquitin-domain protein characterized by a ubiquitin-like domain that regulates the movement of misfolded proteins from the endoplasmic reticulum membrane to proteasome. However, its function in skeletal biology remains unclear. Here, we report that GdX plays a crucial role in skeletal development as mice lacking GdX exhibit skeletal dysplasias, mild kyphosis, and scoliosis. During embryonic stage, GdX knockout mice display decreased bone mineral density and trabecular bone accompanied by delayed osteogenic formation. GdX knockout mice also have blended spine and small body size. At the molecular level, GdX knockout mice showed perturbed expression of osteogenesis-related genes and cartilage developmental genes, indicative of altered differentiation of mesenchymal cell lineage. Collectively, our results uncovered GdX as a novel regulator in bone development and a potential candidate gene for skeletal dysplasias.",
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GdX/UBL4A null mice exhibit mild kyphosis and scoliosis accompanied by dysregulation of osteoblastogenesis and chondrogenesis. / Liang, J.; Li, J.; Fu, Y.; Ren, F.; Xu, J.; Zhou, M.; Li, P.; Feng, H.; Wang, Y.

In: Cell Biochemistry and Function, Vol. 36, No. 3, 01.04.2018, p. 129-136.

Research output: Contribution to journalArticle

TY - JOUR

T1 - GdX/UBL4A null mice exhibit mild kyphosis and scoliosis accompanied by dysregulation of osteoblastogenesis and chondrogenesis

AU - Liang, J.

AU - Li, J.

AU - Fu, Y.

AU - Ren, F.

AU - Xu, J.

AU - Zhou, M.

AU - Li, P.

AU - Feng, H.

AU - Wang, Y.

PY - 2018/4/1

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