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Mutations in both genes underlie human pathologies causing action myoclonus\u2010renal failure syndrome (<jats:styled-content style=\"fixed-case\">AMRF<\/jats:styled-content>) and Gaucher disease (<jats:styled-content style=\"fixed-case\">GD<\/jats:styled-content>), respectively. We now demonstrate that the lumenal acidification mediated by the vacuolar (H<jats:sup>+<\/jats:sup>)\u2010<jats:styled-content style=\"fixed-case\">ATPase<\/jats:styled-content> triggers the dissociation of <jats:styled-content style=\"fixed-case\">LIMP<\/jats:styled-content>\u20102 and <jats:styled-content style=\"fixed-case\">GC<\/jats:styled-content> in late endosomal\/lysosomal compartments. Moreover, we identified a single histidine residue in <jats:styled-content style=\"fixed-case\">LIMP<\/jats:styled-content>\u20102 that is necessary for <jats:styled-content style=\"fixed-case\">LIMP<\/jats:styled-content>\u20102 and <jats:styled-content style=\"fixed-case\">GC<\/jats:styled-content> binding. This residue is in close proximity to a proposed coiled\u2010coil domain, which determines the binding to <jats:styled-content style=\"fixed-case\">GC<\/jats:styled-content> and may function as a critical <jats:styled-content style=\"fixed-case\">pH<\/jats:styled-content> sensor.<\/jats:bold><\/jats:p>","DOI":"10.1111\/j.1600-0854.2012.01372.x","type":"journal-article","created":{"date-parts":[[2012,4,26]],"date-time":"2012-04-26T15:10:32Z","timestamp":1335453032000},"page":"1113-1123","source":"Crossref","is-referenced-by-count":49,"title":["A Critical Histidine Residue Within <scp>LIMP<\/scp>\u20102 Mediates <scp>pH<\/scp>\u00a0Sensitive Binding to Its Ligand \u03b2\u2010Glucocerebrosidase"],"prefix":"10.1111","volume":"13","author":[{"given":"Christina","family":"Zachos","sequence":"first","affiliation":[{"name":"Institut f\u00fcr Biochemie Christian\u2010Albrechts\u2010Universit\u00e4t zu Kiel  Olshausenstrasse 40 D\u201024098 Kiel 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