Article
Structural basis for the wobbler mouse neurodegenerative disorder caused by mutation in the Vps54 subunit of the GARP complex.
Proceedings of the National Academy of Sciences of the United States of America - 20 Jul 2010
Pérez-Victoria F Javier, Abascal-Palacios Guillermo, Tascón Igor, Kajava Andrey, Magadán Javier G, Pioro Erik P, Bonifacino Juan S, Hierro Aitor
Abstract excerpt
The multisubunit Golgi-associated retrograde protein (GARP) complex is required for tethering and fusion of endosome-derived transport vesicles to the trans-Golgi network. Mutation of leucine-967 to glutamine in the Vps54 subunit of GARP is responsible for spinal muscular atrophy in the wobbler mouse, an animal model of amyotrophic lateral sclerosis. The crystal structure at 1.7 A resolution of the mouse Vps54...
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