title: The yeast model for Batten disease: a role for Btn2p in the trafficking of the Golgi-associated vesicular targeting protein, Yif1p.
abstract: Btn2p is a novel coiled coil cytosolic protein in Saccharomyces cerevisiae. We report that Btn2p interacts with Yif1p, a component of a protein complex at the Golgi that functions in ER to Golgi transport. Deletion of Btn2p, btn2-delta, results in mis-localiztion of Yif1p to the vacuole. Therefore, Btn2p may have an apparent role in intracellular trafficking of proteins. Btn2p was originally identified as being up-regulated in a btn1-delta strain, which exhibits dysregulation of vacuolar pH, and this up-regulation of Btn2p was presumed to contribute to maintaining a stable vacuolar pH [Pearce et al. Nat. Genet. 22 (1999) 55]. We propose that up-regulation of Btn2p in btn1-delta is an indicator of altered trafficking within the cell, and as btn1-delta serves as a model for the lysosomal storage disorder Batten disease, that altered intracellular trafficking may contribute to some of the cellular pathological hallmarks of this disease.
authors.affiliation: Center for Aging and Developmental Biology, University of Rochester School of Medicine and Dentistry, Rochester, NY 14642, USA. subrata_chattopadhyay@urmc.rochester.edu
authors.forename: Subrata
authors.initials: S
authors.lastname: Chattopadhyay
authors.forename: Paul M
authors.initials: PM
authors.lastname: Roberts
authors.forename: David A
authors.initials: DA
authors.lastname: Pearce
mesh_terms: D033942:Adaptor Proteins, Vesicular Transport; D026905:Amino Acid Transport Systems; D016213:Cyclins / Q000235:genetics*; D003600:Cytosol / Q000378:metabolism; D004721:Endoplasmic Reticulum / Q000378:metabolism; D017353:Gene Deletion; D015723:Gene Library; D006056:Golgi Apparatus / Q000378:metabolism*; D006801:Humans; D006863:Hydrogen-Ion Concentration; D008562:Membrane Glycoproteins; D018832:Molecular Chaperones; D009472:Neuronal Ceroid-Lipofuscinoses / Q000235:genetics* / Q000378:metabolism; D009474:Neurons / Q000378:metabolism; D010641:Phenotype; D011233:Precipitin Tests; D011485:Protein Binding; D021381:Protein Transport; D012441:Saccharomyces cerevisiae / Q000378:metabolism*; D029701:Saccharomyces cerevisiae Proteins / Q000235:genetics* / Q000378:metabolism / Q000502:physiology*; D020798:Two-Hybrid System Techniques; D033921:Vesicular Transport Proteins; D050888:Heat-Shock Proteins, Small
chemical_list: D033942:Adaptor Proteins, Vesicular Transport; D026905:Amino Acid Transport Systems; D016213:Cyclins; D008562:Membrane Glycoproteins; D018832:Molecular Chaperones; D029701:Saccharomyces cerevisiae Proteins; C473804:BTN2 protein, S cerevisiae; D033921:Vesicular Transport Proteins; D050888:Heat-Shock Proteins, Small; C079895:CLN3 protein, S cerevisiae; C105199:CLN3 protein, human; C489809:YHC3 protein, S cerevisiae; C473803:YIF1 protein, S cerevisiae