title: The origin and targeting of mucosa-associated lymphoid tissue lymphomas.
abstract: OBJECTIVE
  Extranodal mucosa-associated lymphoid tissue (MALT lymphoma) is a distinct clinical-pathological entity that can be distinguished from other lymphomas by a number of unique features, including their location in various extranodal sites, being preceded by chronic inflammatory or infection processes; a characteristic histopathological picture; and the presence of exclusive chromosomal translocations which increase MALT1 proteolytic activity to promote constitutive NF-κB signaling and eventually drive lymphomagenesis.
  
  
  RESULTS
  This review explores the major molecular and cellular events that participate in MALT lymphoma pathogenesis, focusing on gastric MALT lymphoma as a model of chronic inflammation-induced tumor development. In addition, the pivotal roles of activated MALT1 protease, its substrate TNFAIP3/A20, and the MyD88 adaptor protein in abnormally triggering downstream NF-κB pathway are overviewed. These new insights provide a mechanistic basis for using novel therapies targeting MALT1 protease or IRAK4 kinase activities. Finally, the putative cellular origin of MALT lymphomas is also discussed.
  
  
  CONCLUSIONS
  Over the last decade, unraveling the biological complexity of MALT lymphomas has shed light on the fundamental cellular and molecular aspects of the disease that are to be translated into clinical diagnostics and therapy.
authors.affiliation: Division of Hematological Oncology, Center for Applied Medical Research, University of Navarra, Pamplona, Spain.
authors.forename: Jose A
authors.initials: JA
authors.lastname: Martinez-Climent
mesh_terms: D000818:Animals; D020169:Caspases / Q000235:genetics; D002908:Chronic Disease; D006801:Humans; D007249:Inflammation / Q000150:complications; D018442:Lymphoma, B-Cell, Marginal Zone / Q000188:drug therapy / Q000209:etiology* / Q000378:metabolism; D058990:Molecular Targeted Therapy; D000074884:Mucosa-Associated Lymphoid Tissue Lymphoma Translocation 1 Protein; D009154:Mutation; D016328:NF-kappa B / Q000378:metabolism; D009363:Neoplasm Proteins / Q000235:genetics; D015398:Signal Transduction; D014178:Translocation, Genetic
chemical_list: D016328:NF-kappa B; D009363:Neoplasm Proteins; D020169:Caspases; C400109:MALT1 protein, human; D000074884:Mucosa-Associated Lymphoid Tissue Lymphoma Translocation 1 Protein