Global defects in collagen secretion in a Mia3/TANGO1 knockout mouse

被引:152
作者
Wilson, Deanna G. [1 ]
Phamluong, Khanhky [1 ]
Li, Li [2 ]
Sun, Mei [3 ]
Cao, Tim C. [4 ]
Liu, Peter S. [5 ]
Modrusan, Zora [1 ]
Sandoval, Wendy N. [5 ]
Rangell, Linda [3 ]
Carano, Richard A. D. [4 ]
Peterson, Andrew S. [1 ]
Solloway, Mark J. [1 ]
机构
[1] Genentech Inc, Dept Mol Biol, San Francisco, CA 94080 USA
[2] Genentech Inc, Dept Bioinformat & Computat Biol, San Francisco, CA 94080 USA
[3] Genentech Inc, Dept Pathol, San Francisco, CA 94080 USA
[4] Genentech Inc, Dept Res Oncol, San Francisco, CA 94080 USA
[5] Genentech Inc, Dept Prot Chem, San Francisco, CA 94080 USA
关键词
MELANOMA INHIBITORY-ACTIVITY; ENDOPLASMIC-RETICULUM; MALIGNANT-MELANOMA; ALPHA-1; CHAIN; N-PROTEINASE; GROWTH-PLATE; MIA/CD-RAP; CELL-DEATH; COPII; GENE;
D O I
10.1083/jcb.201007162
中图分类号
Q2 [细胞生物学];
学科分类号
071009 ; 090102 ;
摘要
Melanoma inhibitory activity member 3 (MIA3/TANGO1/ARNT) is an evolutionarily conserved endoplasmic reticulum resident transmembrane protein. Recent in vitro studies have shown that it is required for the loading of collagen VII, but not collagen I, into COPII-coated transport vesicles. In this paper, we show that mice lacking Mia3 are defective for the secretion of numerous collagens, including collagens I, II, III, IV, VII, and IX, from chondrocytes, fibroblasts, endothelial cells, and mural cells. Collagen deposition by these cell types is abnormal, and extracellular matrix composition is compromised. These changes are associated with intracellular accumulation of collagen and the induction of a strong unfolded protein response, primarily within the developing skeleton. Chondrocyte maturation and bone mineralization are severely compromised in Mia3-null embryos, leading to dwarfism and neonatal lethality. Thus, Mia3's role in protein secretion is much broader than previously realized, and it may, in fact, be required for the efficient secretion of all collagen molecules in higher organisms.
引用
收藏
页码:935 / 951
页数:17
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