title: Golgi disruption and early embryonic lethality in mice lacking USO1.
abstract: Golgins are a family of long rod-like proteins characterized by the presence of central coiled-coil domains. Members of the golgin family have important roles in membrane trafficking, where they function as tethering factors that capture transport vesicles and facilitate membrane fusion. Golgin family members also have essential roles in maintaining the organization of the Golgi apparatus. Knockdown of individual golgins in cultured cells resulted in the disruption of the Golgi structure and the dispersal of Golgi marker proteins throughout the cytoplasm. However, these cellular phenotypes have not always been recapitulated in vivo. For example, embryonic development proceeds much further than expected and Golgi disruption was observed in only a subset of cell types in mice lacking the ubiquitously expressed golgin GMAP-210. Cell-type specific functional compensation among golgins may explain the absence of global cell lethality when a ubiquitously expressed golgin is missing. In this study we show that functional compensation does not occur for the golgin USO1. Mice lacking this ubiquitously expressed protein exhibit disruption of Golgi structure and early embryonic lethality, indicating that USO1 is indispensable for early embryonic development.
authors.affiliation: Orthopaedic Research Laboratories, Department of Orthopaedic Surgery, Boston Children's Hospital, Boston, Massachusetts, United States of America.
authors.forename: Susie
authors.initials: S
authors.lastname: Kim
authors.forename: Adele
authors.initials: A
authors.lastname: Hill
authors.forename: Matthew L
authors.initials: ML
authors.lastname: Warman
authors.forename: Patrick
authors.initials: P
authors.lastname: Smits
mesh_terms: D000483:Alleles; D000818:Animals; D020964:Embryo Loss / Q000235:genetics* / Q000378:metabolism; D004622:Embryo, Mammalian; D005091:Exons; D005260:Female; D005347:Fibroblasts / Q000378:metabolism / Q000473:pathology; D006056:Golgi Apparatus / Q000235:genetics* / Q000378:metabolism / Q000473:pathology; D000077614:Golgi Matrix Proteins; D006579:Heterozygote; D006720:Homozygote; D007438:Introns; D008297:Male; D051379:Mice; D018345:Mice, Knockout; D011247:Pregnancy; D061251:Primary Cell Culture; D021381:Protein Transport; D013997:Time Factors; D033921:Vesicular Transport Proteins / Q000172:deficiency / Q000235:genetics*
chemical_list: D000077614:Golgi Matrix Proteins; D033921:Vesicular Transport Proteins; C091580:vesicular transport factor p115