title: An immunohistochemical study of placental syncytiotrophoblasts in neonatal hemochromatosis.
abstract: Neonatal hemochromatosis (NH) is a rare neonatal disorder that results in liver cirrhosis with hemosiderin deposition in the liver and other organs, similarly to hereditary hemochromatosis. Excess iron is transferred from the mother to fetus through the placenta in NH. We examined the expression of iron metabolism-related substances in placental syncytiotrophoblasts (STB) by immunostaining to clarify how the transfer of iron through STB increases in NH.
  
  
  
  Immunostaining was performed using formalin-fixed, paraffin-embedded sections of placentae from three NH cases, four gestational age-matched controls, and, depending on the antibody examined, five to seven full-term controls. The reactivity of immunostaining was assessed by averages of scores assigned by 3 researchers.
  
  
  
  On the microvillar surface of STB, the reactions of the antibodies against transferrin receptor 1 (TFR1), transferrin, ferritin, hepcidin, ferroportin, divalent metal transporter-1 (DMT1), hephaestin, and HFE were stronger in NH than in controls. In the cytoplasm, the reactions of antibodies against TFR1, transferrin, ferritin, hepcidin, DMT1, hephaestin, HFE, and ZIP 14 were stronger in NH than in gestational age-matched controls. Among these reactions, those of anti-TFR1 antibody on the surface of STB in NH was especially marked.
  
  
  
  In the placenta of NH, increases in expressions of TFR1, transferrin, and ferritin of which those of TFR1 were especially marked, reflect increased iron influx from the mother to fetus. The hepcidin observed on the surface and in the cytoplasm of STB of NH is suggested to be from the mother, possibly to compensate for the decreased fetal liver-derived hepcidin.
authors.affiliation: Inflammation Pathology, Department of Pathology and Host Defense, Faculty of Medicine, Kagawa University, 1750-1, Ikenobe, Mikicho, Kitagun, Kagawa Prefecture 761-0793, Japan.
authors.forename: Aiko
authors.initials: A
authors.lastname: Shimono
authors.affiliation: Inflammation Pathology, Department of Pathology and Host Defense, Faculty of Medicine, Kagawa University, 1750-1, Ikenobe, Mikicho, Kitagun, Kagawa Prefecture 761-0793, Japan.
authors.forename: Yuko
authors.initials: Y
authors.lastname: Imoto
authors.affiliation: Inflammation Pathology, Department of Pathology and Host Defense, Faculty of Medicine, Kagawa University, 1750-1, Ikenobe, Mikicho, Kitagun, Kagawa Prefecture 761-0793, Japan. Electronic address: profsaka@med.kagawa-u.ac.jp.
authors.forename: Haruhiko
authors.initials: H
authors.lastname: Sakamoto
authors.affiliation: Inflammation Pathology, Department of Pathology and Host Defense, Faculty of Medicine, Kagawa University, 1750-1, Ikenobe, Mikicho, Kitagun, Kagawa Prefecture 761-0793, Japan.
authors.forename: Yoichi
authors.initials: Y
authors.lastname: Chiba
authors.affiliation: Inflammation Pathology, Department of Pathology and Host Defense, Faculty of Medicine, Kagawa University, 1750-1, Ikenobe, Mikicho, Kitagun, Kagawa Prefecture 761-0793, Japan.
authors.forename: Koichi
authors.initials: K
authors.lastname: Matsumoto
authors.affiliation: Inflammation Pathology, Department of Pathology and Host Defense, Faculty of Medicine, Kagawa University, 1750-1, Ikenobe, Mikicho, Kitagun, Kagawa Prefecture 761-0793, Japan.
authors.forename: Machi
authors.initials: M
authors.lastname: Kawauchi
authors.affiliation: Department of Pediatrics, Faculty of Medicine, Kagawa University, 1750-1, Ikenobe, Mikicho, Kitagun, Kagawa Prefecture 761-0793, Japan.
authors.forename: Takashi
authors.initials: T
authors.lastname: Kusaka
authors.affiliation: Department of Perinatal Medicine, Faculty of Medicine, Kagawa University, 1750-1, Ikenobe, Mikicho, Kitagun, Kagawa Prefecture 761-0793, Japan.
authors.forename: Hirokazu
authors.initials: H
authors.lastname: Tanaka
authors.affiliation: Department of Perinatal Medicine, Faculty of Medicine, Kagawa University, 1750-1, Ikenobe, Mikicho, Kitagun, Kagawa Prefecture 761-0793, Japan.
authors.forename: Toshiyuki
authors.initials: T
authors.lastname: Hata
authors.affiliation: Division of Diagnostic Pathology, Kagawa University Hospital, 1750-1, Ikenobe, Mikicho, Kitagun, Kagawa Prefecture 761-0793, Japan.
authors.forename: Yoshio
authors.initials: Y
authors.lastname: Kushida
authors.affiliation: Inflammation Pathology, Department of Pathology and Host Defense, Faculty of Medicine, Kagawa University, 1750-1, Ikenobe, Mikicho, Kitagun, Kagawa Prefecture 761-0793, Japan.
authors.forename: Masaki
authors.initials: M
authors.lastname: Ueno
mesh_terms: D000328:Adult; D015703:Antigens, CD / Q000378:metabolism; D027682:Cation Transport Proteins / Q000378:metabolism; D005260:Female; D005293:Ferritins / Q000378:metabolism; D006432:Hemochromatosis / Q000378:metabolism*; D000071020:Hemochromatosis Protein / Q000378:metabolism; D064451:Hepcidins / Q000378:metabolism; D006801:Humans; D007150:Immunohistochemistry; D007501:Iron / Q000378:metabolism*; D008565:Membrane Proteins / Q000378:metabolism; D010920:Placenta / Q000378:metabolism*; D011247:Pregnancy; D011990:Receptors, Transferrin / Q000378:metabolism; D014157:Transcription Factors / Q000378:metabolism; D014168:Transferrin / Q000378:metabolism; D014327:Trophoblasts / Q000378:metabolism*; D055815:Young Adult; D000098742:Ferroportin
chemical_list: D015703:Antigens, CD; C068126:CD71 antigen; D027682:Cation Transport Proteins; C110547:DMRT1 protein; C492164:HEPH protein, human; D000071020:Hemochromatosis Protein; D064451:Hepcidins; D008565:Membrane Proteins; D011990:Receptors, Transferrin; D014157:Transcription Factors; D014168:Transferrin; D000098742:Ferroportin; D005293:Ferritins; D007501:Iron
keywords: Ferroportin; Hepcidin; Immunohistochemsitry; Iron; Liver cirrhosis; Transferrin receptor 1