title: Cardiac amyloidosis induces up-regulation of Deleted in Malignant Brain Tumors 1 (DMBT1).
abstract: BACKGROUND
  Amyloidosis is a life-threatening protein misfolding disease and affects cardiac tissue, leading to heart failure, myocardial ischemia and arrhythmia. Amyloid deposits result in oxidative stress, inflammation and apoptosis. The purpose of this study was to examine the role of innate defense components, i.e., Deleted in Malignant Brain Tumors 1 (DMBT1) and the complement system, in different types of cardiac amyloidosis.
  
  
  METHODS
  Expression of DMBT1 and of the complement proteins C1q, C3d and C4d in cardiac specimens of patients with different types of amyloidosis were determined by immunohistochemistry and correlated with amyloid deposits stained by Congo red dye.
  
  
  RESULTS
  Strong DMBT1 staining adjacent to amyloid deposits was detected in different amyloidosis types, depending on the extent of the deposits. DMBT1 is localized in the endomysium and perimysium, in the endocardium, in the myocytes and in endothelial cells of affected transmural vessels. C1q, C3d and C4d were detected in the amyloid deposits but also in the endomysium and perimysium, in some myocytes, in endothelial cells, in the endocardium, and around the amyloid deposits.
  
  
  CONCLUSIONS
  Up-regulated DMBT1 and complement activation in cardiac amyloidosis may be part of the activated pathways induced by protein aggregation and the consecutive inflammatory reaction.
authors.affiliation: Division of Neonatology, Department of Pediatrics, University of Heidelberg, Im Neuenheimer Feld 430, 69120 Heidelberg, Germany. Hanna.Mueller@med.uni-heidelberg.de
authors.forename: Hanna
authors.initials: H
authors.lastname: Müller
authors.forename: Marcus
authors.initials: M
authors.lastname: Renner
authors.forename: Frank
authors.initials: F
authors.lastname: Bergmann
authors.forename: Gunhild
authors.initials: G
authors.lastname: Mechtersheimer
authors.forename: Christel
authors.initials: C
authors.lastname: Weiss
authors.forename: Johannes
authors.initials: J
authors.lastname: Poeschl
authors.forename: Burkhard M
authors.initials: BM
authors.lastname: Helmke
authors.forename: Jan
authors.initials: J
authors.lastname: Mollenhauer
mesh_terms: D000368:Aged; D000686:Amyloidosis / Q000378:metabolism* / Q000473:pathology; D002135:Calcium-Binding Proteins; D003167:Complement Activation; D004268:DNA-Binding Proteins; D005260:Female; D006331:Heart Diseases / Q000378:metabolism* / Q000473:pathology; D006801:Humans; D007150:Immunohistochemistry; D008297:Male; D008875:Middle Aged; D011956:Receptors, Cell Surface / Q000096:biosynthesis*; D025521:Tumor Suppressor Proteins; D015854:Up-Regulation
chemical_list: D002135:Calcium-Binding Proteins; C108174:DMBT1 protein, human; D004268:DNA-Binding Proteins; D011956:Receptors, Cell Surface; D025521:Tumor Suppressor Proteins