title: Traffic jams in fish bones: ER-to-Golgi protein transport during zebrafish development.
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.
authors.affiliation: Department of Medicine, Division of Genetic Medicine, Vanderbilt University Medical Center, Nashville, TN, USA.
authors.forename: David B
authors.initials: DB
authors.lastname: Melville
authors.forename: Ela W
authors.initials: EW
authors.lastname: Knapik
mesh_terms: D000818:Animals; D022181:COP-Coated Vesicles / Q000235:genetics / Q000378:metabolism*; D002352:Carrier Proteins / Q000378:metabolism; D004625:Embryo, Nonmammalian; D004721:Endoplasmic Reticulum / Q000378:metabolism*; D018507:Gene Expression Regulation, Developmental; D006056:Golgi Apparatus / Q000378:metabolism*; D006801:Humans; D021381:Protein Transport; D029701:Saccharomyces cerevisiae Proteins / Q000378:metabolism; D033921:Vesicular Transport Proteins / Q000235:genetics / Q000378:metabolism*; D015027:Zebrafish / Q000196:embryology / Q000378:metabolism; D029961:Zebrafish Proteins / Q000235:genetics / Q000378:metabolism*
chemical_list: D002352:Carrier Proteins; D029701:Saccharomyces cerevisiae Proteins; D033921:Vesicular Transport Proteins; D029961:Zebrafish Proteins