title: Golgi pH, its regulation and roles in human disease.
abstract: Most organelles within the exocytic and endocytic pathways typically acidify their interiors, a phenomenon that is known to be crucial for their optimal functioning in eukaryotic cells. This review highlights recent advances in our understanding of how Golgi acidity is maintained and regulated, and how its misregulation contributes to organelle dysfunction and disease. Both its biosynthetic products (glycans) and protein-sorting events are highly sensitive to changes in Golgi luminal pH and are affected in certain human disease states such as cancers and cutis laxa. Other potential disease states that are caused by, or are associated with, Golgi pH misregulation will also be discussed.
authors.affiliation: Department of Biochemistry, University of Oulu, Oulu, Finland.
authors.forename: Antti
authors.initials: A
authors.lastname: Rivinoja
authors.forename: Francois M
authors.initials: FM
authors.lastname: Pujol
authors.forename: Antti
authors.initials: A
authors.lastname: Hassinen
authors.forename: Sakari
authors.initials: S
authors.lastname: Kellokumpu
mesh_terms: D003483:Cutis Laxa / Q000209:etiology / Q000503:physiopathology; D003550:Cystic Fibrosis / Q000209:etiology / Q000503:physiopathology; D004194:Disease / Q000209:etiology*; D004705:Endocytosis / Q000502:physiology; D005057:Eukaryotic Cells / Q000378:metabolism* / Q000502:physiology; D005089:Exocytosis / Q000502:physiology; D006031:Glycosylation; D006056:Golgi Apparatus / Q000378:metabolism* / Q000502:physiology; D006801:Humans; D006863:Hydrogen-Ion Concentration; D009369:Neoplasms / Q000209:etiology / Q000503:physiopathology; D015388:Organelles / Q000378:metabolism* / Q000502:physiology; D016506:Pemphigus, Benign Familial / Q000209:etiology / Q000503:physiopathology