PUBLICATION

Sec13 safeguards the integrity of the endoplasmic reticulum and organogenesis of the digestive system in zebrafish

Authors
Niu, X., Gao, C., Jan Lo, L., Luo, Y., Meng, C., Hong, J., Hong, W., and Peng, J.
ID
ZDB-PUB-120529-7
Date
2012
Source
Developmental Biology   367(2): 197-207 (Journal)
Registered Authors
Gao, Chuan, Peng, Jinrong
Keywords
Sec13, COPII complex, digestive organs, liver development, zebrafish
MeSH Terms
  • Hepatocytes/cytology
  • Hepatocytes/metabolism
  • Animals
  • Morpholinos/genetics
  • Cell Differentiation
  • Base Sequence
  • Gene Expression Regulation, Developmental
  • Intestinal Mucosa/cytology
  • Intestinal Mucosa/embryology
  • Intestinal Mucosa/metabolism
  • Microscopy, Electron, Transmission
  • Endoplasmic Reticulum/metabolism
  • Mutation
  • Unfolded Protein Response
  • Cell Cycle Checkpoints
  • Amino Acid Sequence
  • RNA Splicing/genetics
  • Organogenesis
  • Apoptosis
  • Chondrocytes/cytology
  • Chondrocytes/metabolism
  • Zebrafish/embryology*
  • Zebrafish/genetics
  • Zebrafish/metabolism*
  • COP-Coated Vesicles/genetics
  • COP-Coated Vesicles/metabolism*
  • Gene Knockdown Techniques
  • Digestive System/embryology*
  • Digestive System/metabolism*
  • Molecular Sequence Data
  • Zebrafish Proteins/antagonists & inhibitors
  • Zebrafish Proteins/genetics
  • Zebrafish Proteins/metabolism*
(all 33)
PubMed
22609279 Full text @ Dev. Biol.
Abstract

The Sec13-Sec31 heterotetramer serves as the outer coat in the COPII complex, which mediates protein trafficking from the endoplasmic reticulum (ER) to the Golgi apparatus. Although it has been studied in depth in yeast and cultured cells, the role of COPII in organogenesis in a multicellular organism has not. We report here that a zebrafish sec13sq198 mutant, which exhibits a phenotype of hypoplastic digestive organs, has a mutation in the sec13 gene. The mutant gene encodes a carboxyl-terminus-truncated Sec13 that loses its affinity to Sec31a, which leads to disintegration of the ER structure in various differentiated cells in sec13sq198, including chondrocytes, intestinal epithelial cells and hepatocytes. Disruption of the ER structure activates an unfolded protein response that eventually causes the cells to undergo cell-cycle arrest and cell apoptosis, which arrest the growth of developing digestive organs in the mutant. Our data provide the first direct genetic evidence that COPII function is essential for the organogenesis of the digestive system.

Genes / Markers
Figures
Figure Gallery (21 images) / 3
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Expression
Phenotype
Mutations / Transgenics
Allele Construct Type Affected Genomic Region
sq198
    Point Mutation
    1 - 1 of 1
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    Human Disease / Model
    No data available
    Sequence Targeting Reagents
    Target Reagent Reagent Type
    sec13MO3-sec13MRPHLNO
    sec31aMO1-sec31aMRPHLNO
    1 - 2 of 2
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    Fish
    Antibodies
    Name Type Antigen Genes Isotypes Host Organism
    Ab1-sec13Mouse
    Ab1-sec31aRabbit
    1 - 2 of 2
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    Orthology
    1 - 6 of 6
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    Engineered Foreign Genes
    No data available
    Mapping
    No data available