title: ROMO1 regulates RedOx states and serves as an inducer of NF-κB-driven EMT factors in Fanconi anemia.
abstract: Fanconi anemia (FA) is a rare genetic disorder associated with a bone-marrow failure, genome instability, hypersensitivity to DNA crosslinking agents and a predisposition to cancer. Mutations have been documented in 16 FA genes that participate in the FA-BRCA DNA repair pathway, a fundamental pathway in the development of the disease and the presentation of its symptoms. Besides the well-established role of FA genes in DNA damage and repair pathways, recent reports have revealed an overproduction of epithelial to mesenchymal transition (EMT) factors via a NF-κB-dependent mechanism that results in the proliferation of neighboring tumor cells and FA cells have also been shown to possess damaged mitochondria, accompanied by altered RedOx pathways. This study has focused on reactive oxygen species Modulator-1 (ROMO1), an oncomarker and mitochondrial membrane protein, which is known to be associated with cancer growth and in the modulation of RedOx states in some cancer models. Here, we reveal the role of ROMO1 and demonstrate its link in regulating RedOx states and in the activation of NF-κB-dependent EMT factors in FA.
authors: Stem cell and Molecular biology Laboratory, Department of Biotechnology, Indian Institute of Technology Madras, Chennai, India.
authors: Pavithra
authors: P
authors: Shyamsunder
authors: Stem cell and Molecular biology Laboratory, Department of Biotechnology, Indian Institute of Technology Madras, Chennai, India.
authors: Rama S
authors: RS
authors: Verma
authors: Novosibirsk Institute of Molecular Biology and Biophysics, Russia; Medical Biology Centre, School of Biological Sciences, Queen's University Belfast, 97 Lisburn Road, Belfast BT9 7BL, UK. Electronic address: lyakhovich@gmail.com.
authors: Alex
authors: A
authors: Lyakhovich
mesh_terms: D017209:Apoptosis; D015153:Blotting, Western; D002448:Cell Adhesion; D002465:Cell Movement; D049109:Cell Proliferation; D002478:Cells, Cultured; D003114:Colony-Forming Units Assay; D058750:Epithelial-Mesenchymal Transition; D005199:Fanconi Anemia / Q000235:genetics / Q000378:metabolism / Q000473:pathology*; D006801:Humans; D008565:Membrane Proteins / Q000037:antagonists & inhibitors / Q000235:genetics / Q000378:metabolism*; D024101:Mitochondrial Proteins / Q000037:antagonists & inhibitors / Q000235:genetics / Q000378:metabolism*; D016328:NF-kappa B / Q000235:genetics / Q000378:metabolism*; D010084:Oxidation-Reduction; D010101:Oxygen Consumption; D012333:RNA, Messenger / Q000235:genetics; D034741:RNA, Small Interfering / Q000235:genetics; D017382:Reactive Oxygen Species / Q000378:metabolism; D060888:Real-Time Polymerase Chain Reaction; D020133:Reverse Transcriptase Polymerase Chain Reaction
chemical_list: D008565:Membrane Proteins; D024101:Mitochondrial Proteins; D016328:NF-kappa B; D012333:RNA, Messenger; D034741:RNA, Small Interfering; C513338:ROMO1 protein, human; D017382:Reactive Oxygen Species
keywords: EMT; Fanconi anemia; Mitochondrial dysfunction; NF-κB; Oxidative stress; ROMO1