Abstract
Extracellular matrix (ECM) proteins, cell adhesion molecules, cytokines, morphogens and membrane receptors are synthesized in the ER and transported through the Golgi complex to the cell surface and the extracellular space. The first leg in this journey from the ER to Golgi is facilitated by the Coat Protein II (COPII) vesicular carriers. Genetic defects in genes encoding various COPII components cause a broad spectrum of human diseases, from anemia to skeletal deformities. Here, we summarize our findings in zebrafish and discuss how mutations in COPII elements may cause specific cellular and developmental defects.
Publication types
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Research Support, N.I.H., Extramural
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Research Support, Non-U.S. Gov't
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Review
MeSH terms
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Animals
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COP-Coated Vesicles / genetics
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COP-Coated Vesicles / metabolism*
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Carrier Proteins / metabolism
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Embryo, Nonmammalian
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Endoplasmic Reticulum / metabolism*
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Gene Expression Regulation, Developmental
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Golgi Apparatus / metabolism*
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Humans
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Protein Transport
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Saccharomyces cerevisiae Proteins / metabolism
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Vesicular Transport Proteins / genetics
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Vesicular Transport Proteins / metabolism*
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Zebrafish / embryology
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Zebrafish / metabolism
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Zebrafish Proteins / genetics
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Zebrafish Proteins / metabolism*
Substances
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Carrier Proteins
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Saccharomyces cerevisiae Proteins
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Vesicular Transport Proteins
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Zebrafish Proteins