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PMID:21606205

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Citation

Wilson, DG, Phamluong, K, Li, L, Sun, M, Cao, TC, Liu, PS, Modrusan, Z, Sandoval, WN, Rangell, L, Carano, RA, Peterson, AS and Solloway, MJ (2011) Global defects in collagen secretion in a Mia3/TANGO1 knockout mouse. J. Cell Biol. 193:935-51

Abstract

Melanoma inhibitory activity member 3 (MIA3/TANGO1) [corrected] 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.

Links

PubMed PMC3105544 Online version:10.1083/jcb.201007162

Keywords

Animals; Aryl Hydrocarbon Receptor Nuclear Translocator/deficiency; Aryl Hydrocarbon Receptor Nuclear Translocator/genetics; Cells, Cultured; Collagen/metabolism; Collagen/secretion; Mice; Mice, Inbred C57BL; Mice, Knockout

Significance

Annotations

Gene product Qualifier GO Term Evidence Code with/from Aspect Extension Notes Status

MOUSE:SERPH

located_in

GO:0005783: endoplasmic reticulum

ECO:0000314: direct assay evidence used in manual assertion

C

  • part_of:(CL:0000138)

Seeded From UniProt

complete

MOUSE:CALX

located_in

GO:0005783: endoplasmic reticulum

ECO:0000314: direct assay evidence used in manual assertion

C

  • part_of:(CL:0000138)

Seeded From UniProt

complete

MOUSE:TGO1

acts_upstream_of_or_within

GO:0030199: collagen fibril organization

ECO:0000315: mutant phenotype evidence used in manual assertion

MGI:MGI:5007248

P

Seeded From UniProt

complete

MOUSE:TGO1

acts_upstream_of_or_within

GO:0002063: chondrocyte development

ECO:0000315: mutant phenotype evidence used in manual assertion

MGI:MGI:5007248

P

Seeded From UniProt

complete

MOUSE:TGO1

acts_upstream_of_or_within

GO:0030501: positive regulation of bone mineralization

ECO:0000315: mutant phenotype evidence used in manual assertion

MGI:MGI:5007248

P

Seeded From UniProt

complete

MOUSE:TGO1

located_in

GO:0005789: endoplasmic reticulum membrane

ECO:0000314: direct assay evidence used in manual assertion

C

  • part_of:(CL:0000138)

Seeded From UniProt

complete

MOUSE:GOGA2

located_in

GO:0005801: cis-Golgi network

ECO:0000314: direct assay evidence used in manual assertion

C

  • part_of:(CL:0000138)

Seeded From UniProt

complete

MOUSE:GOGA2

part_of

GO:0005801: cis-Golgi network

ECO:0000314: direct assay evidence used in manual assertion

C

part_of:(CL:0000138)

Seeded From UniProt

complete

Notes

See also

References

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