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Analysis of TERE1 (aka UBIAD1) has shown it is a prenyltransferase enzyme in the natural bio\u2010synthetic pathways for both vitamin K\u20102 and COQ10 production and exhibits multiple subcellular localizations including mitochondria, endoplasmic reticulum, and golgi. Vitamin K\u20102 is involved in mitochondrial electron transport, SXR nuclear hormone receptor signaling and redox cycling: together these functions may form the basis for tumor suppressor function. To gain further insight into mechanisms of growth suppression and enzymatic regulation of TERE1 we isolated TERE1 associated proteins and identified the WD40 repeat, mitochondrial protein TBL2. We examined whether disease specific mutations in TERE1 affected interactions with TBL2 and the role of each protein in altering mitochondrial function, ROS\/RNS production and SXR target gene regulation. Biochemical binding assays demonstrated a direct, high affinity interaction between TERE1 and TBL2 proteins; TERE1 was localized to both mitochondrial and non\u2010mitochondrial membranes whereas TBL2 was predominantly mitochondrial; multiple independent single amino acid substitutions in TERE1 which cause a human hereditary corneal disease reduced binding to TBL2 strongly suggesting the relevance of this interaction. Ectopic TERE1 expression elevated mitochondrial trans\u2010membrane potential, oxidative stress, NO production, and activated SXR targets. A TERE1\u2010TBL2 complex likely functions in oxidative\/nitrosative stress, lipid metabolism, and SXR signaling pathways in its role as a tumor suppressor. J. Cell. Biochem. 114: 2170\u20132187, 2013. \u00a9 2013 Wiley Periodicals, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/jcb.24567","type":"journal-article","created":{"date-parts":[[2013,4,8]],"date-time":"2013-04-08T11:16:49Z","timestamp":1365419809000},"page":"2170-2187","source":"Crossref","is-referenced-by-count":23,"title":["The TERE1 protein interacts with mitochondrial TBL2: Regulation of trans\u2010membrane potential, ROS\/RNS and SXR target genes"],"prefix":"10.1002","volume":"114","author":[{"given":"William J.","family":"Fredericks","sequence":"first","affiliation":[{"name":"Division of Urology, Department of Surgery University of Pennsylvania and Veterans Affairs Medical Center Philadelphia Philadelphia Pennsylvania 19104"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Terry","family":"McGarvey","sequence":"additional","affiliation":[{"name":"Department of Anatomy Kirksville Osteopathic Medical School Kirksville Missouri 63501"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Huiyi","family":"Wang","sequence":"additional","affiliation":[{"name":"Division of Urology, Department of Surgery University of Pennsylvania and Veterans Affairs Medical Center Philadelphia Philadelphia Pennsylvania 19104"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yongmu","family":"Zheng","sequence":"additional","affiliation":[{"name":"Division of Urology, Department of Surgery University of Pennsylvania and Veterans Affairs Medical Center Philadelphia Philadelphia Pennsylvania 19104"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Nathaniel J.","family":"Fredericks","sequence":"additional","affiliation":[{"name":"Division of Urology, Department of Surgery University of Pennsylvania and Veterans Affairs Medical Center Philadelphia Philadelphia Pennsylvania 19104"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hankun","family":"Yin","sequence":"additional","affiliation":[{"name":"Division of Urology, Department of Surgery University of Pennsylvania and Veterans Affairs Medical Center Philadelphia Philadelphia Pennsylvania 19104"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Li\u2010Ping","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Pathology and Laboratory Medicine University of Pennsylvania Medical Center Philadelphia Pennsylvania 19104"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Wayland","family":"Hsiao","sequence":"additional","affiliation":[{"name":"Department of Urology Weill Cornell Medical College New York New York 10065"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rob","family":"Lee","sequence":"additional","affiliation":[{"name":"Department of Otorhinolaryngology University of Pennsylvania and Veterans Affairs Medical Center Philadelphia Philadelphia Pennsylvania 19104"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jayne S.","family":"Weiss","sequence":"additional","affiliation":[{"name":"Departments of Ophthalmology and Eye Center Louisiana State University New Orleans Louisiana"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael L.","family":"Nickerson","sequence":"additional","affiliation":[{"name":"Cancer and Inflammation Program, National Cancer Institute Frederick Maryland 21702\u20101201"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Howard S.","family":"Kruth","sequence":"additional","affiliation":[{"name":"Section of Experimental Atherosclerosis National Institutes of Health Bethesda Maryland"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Frank J.","family":"RauscherIII","sequence":"additional","affiliation":[{"name":"The Wistar Institute Philadelphia Pennsylvania 19104"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"S. 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